bluuebunny/crossref_metadata_2025 huggingface dataset:
RMRP mutations in Japanese patients with cartilage‐hair hypoplasia
Authors: Nakashima Eiji , Mabuchi Akihiko , Kashimada Kenichi , Onishi Toshikazu , Zhang Junwei , Ohashi Hirofumi , Nishimura Gen , Ikegawa Shiro
Published: November 2003

We examined 12 Japanese patients with metaphyseal chondrodysplasia (MCD) for mutations in the ribonuclease mitochondrial RNA processing gene (RMRP), and identified four novel mutations in two patients with typical and atypical cartilage‐hair hypoplasia (CHH), a form of MCD characterized by extra‐skeletal manifestations including hypoplastic hair and defective immunity. A patient with typical CHH had a 17‐bp duplication at +3 and a de novo 182G > A. The other patient with atypical CHH had a 17‐bp insertion at −20 and a 218A > G. Expression analysis revealed that the allele with this insertion mutation in the promoter region silenced the gene. Spectrum analysis of the mutations and polymorphisms in RMRP showed marked difference between the Japanese and other ethnic groups. Such ethnic and phenotypic difference should be taken into account in mutation analysis of the gene. © 2003 Wiley‐Liss, Inc.
Patient with trisomy 9p and a hypoplastic left heart with a tricentric chromosome 9
Authors: Morrissette Jennifer J.D. , Laufer‐Cahana Ayala , Medne Livija , Russell Karen L. , Venditti Charles P. , Kline Rochelle , Zackai Elaine H. , Spinner Nancy B.
Published: November 2003

We present a patient with a hypoplastic left heart (HLH), dislocations of the hips and knees, and minor dysmorphic features, who had an abnormal karyotype that resulted in trisomy for 9p and a portion of 9q: 46,((, dic(or tri?)(9)(9pter → 9q34::9q21 → 9pter).ish(WCP9++).ish(D9Z5X4 +/+++). The derivative chromosome consisted of an additional copy of the proximal q arm and p arm attached to 9qter in an inverted fashion. Fluorescence in situ hybridization (FISH) using a chromosome 9 β‐satellite probe revealed three signals on the abnormal chromosome 9, suggesting the presence of 3 pericentromeric regions on the der(9). The 9q subtelomere was present on both the normal and derivative chromosome 9, suggesting that very little material, if any, is deleted. © 2003 Wiley‐Liss, Inc.
Unreported manifestations in two Dutch families with Bartsocas–Papas syndrome
Authors: Veenstra‐Knol Hermine E. , Kleibeuker Arianne , Timmer Albertus , ten Kate Leo P. , van Essen Anthonie J.
Published: November 2003

Bartsocas–Papas syndrome (BPS) is a severe autosomal recessive syndrome characterized by neonatal or intrauterine death in most cases, severe popliteal webbing, oligosyndactyly, genital abnormalities, and typical face with short palpebral fissures, ankyloblepharon, hypoplastic nose, orofacial clefts, and small mouth. Until now at least 23 cases with this syndrome in 11 families were described, mostly from Mediterranean origin. We report on two Dutch families with six affected children having BPS. One of the patients was prenatally diagnosed by ultrasound examination. Additional unreported findings were omphalocele and aplasia of the urethra. The intrafamilial resemblance in severity is of importance for the genetic counseling of families and prenatal detection by ultrasound. We discuss possible pathogenic mechanisms and review similar cases from the literature. © 2003 Wiley‐Liss, Inc.
Incidence of the mucopolysaccharidoses in Western Australia
Authors: Nelson John , Crowhurst June , Carey Bill , Greed Lawrence
Published: November 2003

An epidemiological study of the mucopolysaccharidoses in Western Australia (WA) using multiple ascertainment sources was carried out and the incidence rate for the period 1969–1996 was estimated. An incidence of approximately 1 in 107,000 live births was obtained for MPS IH (Hurler phenotype); 1 in 320,000 live births (1 in 165,000 male live births) for MPS II (Hunter Syndrome); 1 in 58,000 for MPS III (Sanfilippo Syndrome); 1 in 640,000 for MPS IVA (Morquio Syndrome type A), and 1 in 320,000 for MPS VI (Maroteaux–Lamy Syndrome). The overall incidence for all types of mucopolysaccharidosis was approximately 1 in 29,000 live births. A comparison is made with incidence estimates obtained from other published studies. © 2003 Wiley‐Liss, Inc.
X chromosome inactivation patterns in Russell–Silver syndrome patients and their mothers
Authors: Beever Christy L. , Peñaherrera Maria S. , Langlois Sylvie , Robinson Wendy R.
Published: November 2003

Russell–Silver syndrome (RSS) is a disorder characterized by pre‐ and post‐natal growth deficiency, triangular facies, relative macrocephaly, and body asymmetry. Maternal uniparental disomy for chromosome 7 has been found in approximately 10% of RSS cases, while the cause in the remaining cases is unknown. Although most cases of RSS are sporadic, at least 25 families have been reported with familial RSS and inheritance patterns of RSS consistent with an X‐linked dominant mutation. Thus, we hypothesized that skewed X‐chromosome inactivation (XCI) could be increased in some females with RSS as a consequence of a tendency to have the mutant allele on the predominantly active chromosome. Alternatively, unaffected mothers of children with RSS may tend to be skewed due to preferential inactivation of the mutant allele. In support of this last hypothesis, a significant increase in extremely skewed XCI (>95%) was found in mothers of children with RSS (4 of 21, 19%) compared to controls (2 of 101, 2%) (P = 0.008). However, an increase in skewed XCI was also observed in female patients who had unexplained short stature but did not fulfill the criteria for RSS (3 of 17, 18%) (P = 0.02), but not in those diagnosed as RSS (0 of 7, n.s.). Different mechanisms may underlie the increase in skewing in each group, possibly being due to different X‐linked mutations or growth restriction during very early in utero development. © 2003 Wiley‐Liss, Inc.
Costello syndrome and neurological abnormalities
Authors: Delrue Marie‐Ange , Chateil Jean‐François , Arveiler Benoit , Lacombe Didier
Published: November 2003

Costello syndrome is a rare but increasingly recognized syndrome of unknown etiology. Neurological abnormalities are not rare in this syndrome and consist of structural and electrophysiological disorders. Ventricular dilatation is observed in more than 40% of cases. Other reported cerebral anomalies are brain atrophy, Chiari malformation and syringomyelia. Although there is insufficient data to propose strict guidelines, it seams reasonnable to have a low threshold for neuroimaging, in general, and particularly when neurologic signs or symptoms are present. Screening including cerebral MRI and EEG should be proposed after a diagnosis of Costello syndrome. The frequency of testing in such children should be guided by neurological follow‐up. © 2003 Wiley‐Liss, Inc.
Clinical, cytogenetic, and molecular observations in a patient with Pallister‐Killian‐syndrome with an unusual karyotype
Authors: Leube Barbara , Majewski Frank , Gebauer Jürgen , Royer‐Pokora Brigitte
Published: November 2003

Pallister‐Killian syndrome is a clinically recognizable syndrome, usually due to a tissue‐limited mosaicism for a supernumary 12p isochromosome (i12p). Here we report an unusual case with tetrasomy/trisomy/disomy 12p mosaic in fibroblasts and trisomy/disomy 12p mosaic in lymphocytes. The tetrasomy 12p was due to an i12p, the trisomy 12p to a single 12p marker. Both marker chromosomes were investigated with conventional cytogenetic techniques and fluorescent in situ hybridization (FISH). Stability under culturing conditions was studied. DNA‐analysis revealed prezygotic maternal origin of the extra 12p material. Clinically, the patient seems to have less retardation than most patients with Pallister‐Killian syndrome. © 2003 Wiley‐Liss, Inc.
Interstitial 1q25.3‐q31.3 deletion in a boy with mild manifestations
Authors: Höglund Pia , Jalkanen Reetta , Marttinen Eino , Alitalo Tiina
Published: November 2003

We describe a 4‐year‐old boy with an accessory right thumb, short and broad toes, cryptorchidism, micrognathia, abnormally modeled ears, and delayed speech development. The chromosome analysis of patient's peripheral blood lymphocytes by conventional GTG banding demonstrated a small deletion in the long arm of chromosome 1. Confirmation and defined localization of the deleted segment to chromosomal bands 1q25.3‐q31.3 was obtained by high resolution prometaphase analysis. Molecular studies, using a set of polymorphic chromosome 1q specific microsatellite markers, localized the deletion between the markers D1S2127 and D1S1727 on the paternally inherited chromosome 1. The maximum physical distance between these markers is approximately 21 Mb. The previously described two patients with 1q25‐q31 deletions both had severe clinical manifestations, just as the other 10 patients with the proposed “intermediate 1q deletion syndrome,” associated with 1q25‐q32 deletions. Distinct from all these patients, the clinical picture of our patient is markedly milder, i.e., without growth retardation, microcephaly, or clear mental retardation. © 2003 Wiley‐Liss, Inc.
Oculo‐facio‐cardio‐dental syndrome: Skewed X chromosome inactivation in mother and daughter suggest X‐linked dominant Inheritance
Authors: Hedera Peter , Gorski Jerome L.
Published: November 2003

Oculo‐facio‐cardio‐dental syndrome (OFCD) is an uncommon multiple congenital anomaly syndrome that is characterized by congenital cataracts, multiple minor facial dysmorphic features, congenital heart defects, and dental anomalies including canine radiculomegaly and oligodontia. Although most cases of OFCD are sporadic, since all reported OFCD individuals have been female, it has been suggested that OFCD is an X‐linked dominant trait. Here we report two affected female patients with OFCD, a mother and daughter, who both had congenital cataracts, microphthalmia, characteristic dental anomalies, and typical facial dysmorphisms. These features were diagnostic for OFCD; thus, these cases represent the second documented instance of mother‐to‐daughter OFCD transmission. In addition to the clinical features typically seen in OFCD individuals, the affected daughter exhibited several additional congenital anomalies including intestinal malrotation and hypoplastic thumbs. Thus, these cases further define and expand the OFCD clinical phenotype. These two individuals also displayed a skewed pattern of X chromosome inactivation. Together, these data strongly support the hypothesis that OFCD is inherited as an X‐linked dominant condition. © 2003 Wiley‐Liss, Inc.
Idiopathic congenital central hypoventilation syndrome: Analysis of genes pertinent to early autonomic nervous system embryologic development and identification of mutations in PHOX2b
Authors: Weese‐Mayer Debra E. , Berry‐Kravis Elizabeth M. , Zhou Lili , Maher Brion S. , Silvestri Jean M. , Curran Mark E. , Marazita Mary L.
Published: November 2003

Idiopathic congenital central hypoventilation syndrome (CCHS) has been linked to autonomic nervous system dysregulation and/or dysfunction (ANSD) since it was first described in 1970. A genetic basis of CCHS has been proposed because of the reports of four families with two affected children, because of mother–child transmission, and because of a recent report of a polyalanine expansion mutation in PHOX2b in a subset of CCHS subjects. We, therefore, studied genes pertinent to early embryologic development of the ANS including mammalian achaete‐scute homolog‐1 (MASH1), bone morphogenic protein‐2 (BMP2), engrailed‐1 (EN1), TLX3, endothelin converting enzyme‐1 (ECE1), endothelin‐1 (EDN1), PHOX2a, and PHOX2b in 67 probands with CCHS, and gender‐ and ethnicity‐matched controls. No disease‐defining mutations were identified in MASH1, BMP2, EN1, TLX3, ECE1, EDN1, or PHOX2a.The 65/67 CCHS probands (97%) were found to be heterozygous for the exon 3 polyalanine expansion mutation identified previously in PHOX2b. Further, there was an association between repeat mutation length and severity of the CCHS/ANSD phenotype. Of the two probands who did not carry the expansion mutation, one had a nonsense mutation in exon 3 which truncated the protein and the other had no mutation in PHOX2b but had a previously reported EDN3 frameshift point mutation. The polyalanine expansion mutation was not found in any of 67 matched controls. Of 54 available families (including 97 unaffected parents), whose child carried the PHOX2b mutation, 4 parents demonstrated mosaicism for an expansion mutation identical to that seen in the CCHS cases, suggesting that not all mutations in affected probands with unaffected parents are de novo. We also studied four women with CCHS who were heterozygous for the PHOX2b mutation, each with one child. Three of the four children were also affected and had the same mutation, demonstrating autosomal dominant inheritance of the mutation. Assay of the PHOX2b polyalanine repeat mutation represents a highly sensitive and specific technique for confirming the diagnosis of CCHS. Identification of the CCHS mutation will lead to clarification of the phenotype, allow for prenatal diagnosis for parents of CCHS probands and adults with CCHS in future pregnancies, and potentially direct intervention strategies for the treatment of CCHS. © 2003 Wiley‐Liss, Inc.
Correlation of GDF5 and connexin 43 mRNA expression during embryonic development
Authors: Coleman Cynthia M. , Loredo Grace A. , Lo Cecilia W. , Tuan Rocky S.
Published: November 2003

Growth/differentiation factor 5 (GDF5) regulates connexin expression and enhances embryonic chondrogenesis in a gap junction‐dependent manner, suggesting that GDF5 action on developmental skeletogenesis is coordinated with gap junction activities. The results shown here demonstrate concordance between the mRNA expression profiles of GDF5 and the gap junction gene, Cx43, in the mouse embryonic limb, spine, and heart, consistent with coordinated functions for these gene products during developmental organogenesis. Anat Rec Part A 275A:1117–1121, 2003. © 2003 Wiley‐Liss, Inc.
Endothelial nitric oxide synthase gene polymorphisms in giant cell arteritis
Authors: Salvarani Carlo , Casali Bruno , Nicoli Davide , Farnetti Enrico , Macchioni Pierluigi , Catanoso Maria Grazia , Chen Qingquan , Bajocchi Gianluigi , Boiardi Luigi
Published: November 2003

Objective

To examine potential associations of the Glu/Asp298 polymorphism in exon 7 and the 4a/b polymorphism in intron 4 of the endothelial nitric oxide synthase (eNOS) gene with susceptibility to and clinical expression of giant cell arteritis (GCA), particularly in patients with versus those without ischemic complications.

Methods

Ninety‐one consecutive patients with biopsy‐proven GCA, who were residents of Reggio Emilia, Italy, and 133 population‐based controls from the same geographic area were genotyped by polymerase chain reaction and allele‐specific oligonucleotide techniques for eNOS polymorphisms in exon 7 and intron 4. The patients were separated into 2 subgroups according to the presence or absence of ischemic complications (visual loss and/or jaw claudication and/or aortic arch syndrome).

Results

The distribution of the Glu/Asp298 genotype differed significantly between GCA patients and controls (corrected P [Pcorr] = 0.003). Carriers of the Asp298 allele (Asp/Asp or Glu/Asp) were significantly more frequent among the GCA patients than among the controls (Pcorr = 0.0002, odds ratio 3.3, 95% confidence interval 1.7–6.3). The distribution of the 4a/b genotype was similar in GCA patients and controls. No significant associations were found when GCA patients with and without ischemic complications were compared.

Conclusion

Our findings show that the Glu/Asp298 polymorphism of the eNOS gene is associated with GCA susceptibility.
Stereoselective Synthesis of anti‐1,4‐Diols by a BH3⋅THF‐Mediated Rearrangement of 1,2‐Disubstituted Cyclobutenes
Authors: Knapp Kolja M. , Goldfuss Bernd , Knochel Paul
Published: November 2003

A new stereoselective rearrangement of cyclobutylboranes, obtained by the hydroboration of 1,2‐disubstituted cyclobutenes, provides anti‐1,4‐diols with good‐to‐excellent diastereoselectivity. The mechanism of the rearrangement is discussed based on theoretical calculations.
SCR von NO an sulfatierten Zirconiumoxiden in Gegenwart von Methan
Authors: Suprun W. , Schädlich K. , Papp H.
Published: November 2003

No abstracts.
Walzenkompaktieren und Trockengranulieren pharmazeutischer Hilfsstoffe am Beispiel von Magnesiumcarbonat
Authors: Freitag F. , Kleinebudde P.
Published: November 2003

No abstracts.
Aufbau und katalytische Aktivität hierarchisch strukturierter Oxid‐Katalysatoren – Teil 2
Authors: Stelzer J.B. , Habel D. , Pohl M.‐M. , Kosslick H. , Feike E. , Caro J. , Schubert H.
Published: November 2003

No abstracts.
The associative striatum: Organization of cortical projections to the dorsocentral striatum in rats
Authors: Reep Roger L. , Cheatwood Joseph L. , Corwin James V.
Published: November 2003

The dorsocentral striatum (DCS) is the major site of input from medial agranular cortex (AGm) and has been implicated as an associative striatal area that is part of a cortical‐subcortical circuit involved in multimodal spatial functions involving directed attention. Anterograde axonal tracing was used to investigate the spatial organization of corticostriatal projections to DCS. Injections of biotinylated dextran amine were made into several cortical areas known to project to DCS based on retrograde tracing data. These included areas AGm, lateral agranular cortex (AGl), orbital cortex, posterior parietal cortex (PPC), and visual association cortex. We discovered a previously undescribed geometry whereby the projection from AGm is prominent within DCS and the main corticostriatal projections from areas other than AGm are situated around the periphery of DCS: visual association cortex dorsomedially, PPC dorsally, AGl laterally, and orbital cortex ventrally. Each of these cortical projections is also represented by less dense aggregates of terminal labeling within DCS, organized as focal patches and more diffuse labeling. Because these cortical areas are linked by corticocortical connections, the present findings indicate that interconnected cortical areas have convergent terminal fields in the region of DCS. These findings suggest that DCS is a central associative region of the dorsal striatum characterized by a high degree of corticostriatal convergence. J. Comp. Neurol. 467:271–292, 2003. © 2003 Wiley‐Liss, Inc.
Ultrastructural localization of δ‐opioid receptors in the rat caudate‐putamen nucleus during postnatal development: Relation to synaptogenesis
Authors: Wang Hong , Cuzon Verginia C. , Pickel Virginia M.
Published: November 2003

During development, δ‐opioid receptors (DORs) in the rat caudate‐putamen nucleus (CPN) appear later than μ‐opioid receptors (MORs), whose developmental pattern specifically relates to synaptogenesis. We used electron microscopic immunocytochemistry to determine whether there are also age‐related changes in subcellular localization of DORs in the rat CPN. Sections from postnatal day (P) 0–P30 and adult dorsomedial CPN were immunogold‐silver labeled to examine the plasmalemmal and cytoplasmic distribution of these receptors. In addition, immunoperoxidase labeling was used to determine the numerical density of synapses relative to DOR‐labeled profiles. Immunolabeling for DOR was undetectable at P0, light at P5, and dense from P10 onward. The labeling during P5–P10 was mainly localized in somatodendritic profiles but also was readily seen in axon terminals, most of which formed asymmetric synapses with dendrites. From P15, a few immunogold particles were seen in contact with postsynaptic densities in spines, and the proportion of these particles significantly increased in P30 and adult CPN. Other particles were localized in the cytoplasm of dendrites and terminals without significant age‐related changes. Stereological analysis showed that compared with labeled dendritic shafts and spines, labeled axon terminals have a closer correlation with synapse formation. These results are in marked contrast with MORs, which show an age‐related increase in association with dendritic plasma membrane and a good correlation in the developmental pattern of MOR‐labeled spines with synapse formation (Wang et al. [2003] Neuroscience 118:695–708). Together, our results suggest receptor‐type specific roles for endogenous opioids acting at both pre‐ and postsynaptic sides in the developing CPN. J. Comp. Neurol. 467:343–353, 2003. © 2003 Wiley‐Liss, Inc.
Effects of restricted cochlear lesions in adult cats on the frequency organization of the inferior colliculus
Authors: Irvine Dexter R.F. , Rajan Ramesh , Smith Simon
Published: November 2003

Restricted cochlear lesions in adult animals result in plastic changes in the representation of the lesioned cochlea, and thus in the frequency map, in the contralateral auditory cortex and thalamus. To examine the contribution of subthalamic changes to this reorganization, the effects of unilateral mechanical cochlear lesions on the frequency organization of the central nucleus of the inferior colliculus (ICC) were examined in adult cats. Lesions typically resulted in a broad high‐frequency hearing loss extending from a frequency in the range 15–22 kHz. After recovery periods of 2.5–18 months, the frequency organization of ICC contralateral to the lesioned cochlea was determined separately for the onset and late components of multiunit responses to tone‐burst stimuli. For the late response component in all but one penetration through the ICC, and for the onset response component in more than half of the penetrations, changes in frequency organization in the lesion projection zone were explicable as the residue of prelesion responses. In half of the penetrations exhibiting nonresidue type changes in onset‐response frequency organization, the changes appeared to reflect the unmasking of normally inhibited inputs. In the other half it was unclear whether the changes reflected unmasking or a dynamic process of reorganization. Thus, most of the observed changes were explicable as passive consequences of the lesion, and there was limited evidence for plasticity in the ICC. The implications of the data with respect to the primary locus of the changes and to the manner in which they contribute to thalamocortical reorganization are considered. J. Comp. Neurol. 467:354–374, 2003. © 2003 Wiley‐Liss, Inc.
Immunocytochemical evidence for nitric oxide‐ and carbon monoxide‐producing neurons in the stomatogastric nervous system of the crayfish Cherax quadricarinatus
Authors: Christie Andrew E. , Edwards John M. , Cherny Elena , Clason Todd A. , Graubard Katherine
Published: November 2003

Nitric oxide (NO) and carbon monoxide (CO) have been shown to serve neuromodulatory roles in both vertebrates and invertebrates. Here, we use antibodies to their respective biosynthetic enzymes, nitric oxide synthase (NOS) and heme oxygenase 2 (HO‐2), to map the distribution of putative gas‐producing neurons in the stomatogastric nervous system (STNS) of the crayfish Cherax quadricarinatus. In this species, NOS immunolabeling is found in the neuropil of the stomatogastric ganglion (STG). This staining originates from two immunopositive axons that project to the STG through the superior oesophageal and stomatogastric nerves, presumably from cell bodies located in the commissural ganglia (CoGs). HO‐2 immunoreactivity is present in small diameter fibers and varicosities in the periphery of nerves located in the anterior portion of the STNS. This labeling originates from approximately 12 somata in each CoG. Transmission electron microscopy done on the nerves of the anterior STNS shows they contain a neuroendocrine plexus. Collectively, our results indicate that NO‐ and CO‐producing neurons are likely to exist in the crayfish STNS. Moreover, these gases appear to be produced by distinct subsets of the neurons present there. The localization of NO to the STG neuropil suggests that it serves as a locally released modulator or is involved in the local release of other substances within this ganglion. The presence of CO in the neurohemal plexus of the anterior STNS suggests that it serves as a circulating hormone or is involved in the control of neuroendocrine release from this plexus. J. Comp. Neurol. 467:293–306, 2003. © 2003 Wiley‐Liss, Inc.
Mesothoracic ventral unpaired median (mesVUM) neurons in the blowfly Calliphora erythrocephala
Authors: Schlurmann Michael , Hausen Klaus
Published: November 2003

The study describes five ventral unpaired median neurons in the mesothoracic neuromere of the fused thoracic ganglion of Calliphora identified by biocytin staining (mesVUM neurons). The group comprises four efferent neurons and one interneuron which are characterized by a common soma cluster in the ventral midline of the neuromere, bifurcating primary neurites and bilaterally symmetrical arborizations. Respective soma clusters of not‐yet‐identified VUM neurons were also found in the prothoracic, metathoracic, and abdominal neuromeres. The efferent mesVUM neurons are associated with the flight system. Their main arborizations are located in the mesothoracic wing neuropil and their bilateral axons terminate at the flight control muscles, the flight starter muscles, the flight power muscles, or at myocuticular junctions of the latter. In contrast, an association of the interneuron with a particular functional system is not apparent. The arborizations of the neuron are intersegmental and invade all thoracic neuromeres. A further difference between the two types of neurons regards their somatic action potentials, which are overshooting in the efferent neurons and strongly attenuated in the interneuron. Immunocytochemical stainings revealed four clusters of octopamine‐immunoreactive (OA‐IR) somata in the thoracic ganglion, which reside in the same positions as the VUM somata. We regard this as strong evidence that all groups of VUM neurons contain OA‐IR cells and that, in particular, the identified efferent mesVUM neurons are OA‐IR. Our results demonstrate that the mesVUM neurons of Calliphora have similar morphological, electrophysiological, and presumably also immunocytochemical characteristics as the unpaired median neurons of other insects. J. Comp. Neurol. 467:435–453, 2003. © 2003 Wiley‐Liss, Inc.
Architectonics and cortical connections of the upper bank of the superior temporal sulcus in the rhesus monkey: An analysis in the tangential plane
Authors: Padberg Jeffrey , Seltzer Ben , Cusick Catherine G.
Published: November 2003

Area TPO in the upper bank of the superior temporal sulcus (STS) of macaque monkeys is thought to correspond to the superior temporal polysensory (STP) cortex, but has been shown to have neurochemical/connectional subdivisions. To examine directly the relationship between chemoarchitecture and cortical connections of area TPO, the upper bank of the STS was sectioned tangential to the cortical surface. Three subdivisions of area TPO (TPOr, TPOi, and TPOc) were examined with cytochrome oxidase (CO) histochemistry and neurofilament protein (NF) immunoreactivity and architectonic patterns were compared with connections on the same or adjacent sections. Area TPOc, which may partly overlap with the location of the medial superior temporal area MST, exhibited regular patchy staining for CO in layers III/IV and a complementary pattern in the NF stain. Area TPOr, but not TPOi, also had a patchy pattern of complementary staining in CO and neurofilament similar to TPOc, although not as distinct. Tracer injections within cortex including the frontal eye fields (areas 46 and 8) labeled areas TPOc, TPOi, and TPOr. The caudal inferior parietal lobule (IPL) projected to all three areas. The projections from prearcuate and posterior parietal cortices showed both overlap and nonoverlap with each other within TPOc, TPOi, and TPOr. Projections were to all neurochemical components within the subdivisions of TPO. The findings support the parcellation of area TPO into three subdivisions and extend findings of chemoarchitectonic modules within high‐order association cortices. J. Comp. Neurol. 467:418–434, 2003. © 2003 Wiley‐Liss, Inc.
Cellular GDNF delivery promotes growth of motor and dorsal column sensory axons after partial and complete spinal cord transections and induces remyelination
Authors: Blesch Armin , Tuszynski Mark H.
Published: November 2003

Glial cell line‐derived neurotrophic factor (GDNF) is the prototypical member of a growth factor family that signals via the cognate receptors ret and GDNF‐receptor alpha‐1. The latter receptors are expressed on a variety of neurons that project into the spinal cord, including supraspinal neurons, dorsal root ganglia, and local neurons. Although effects of GDNF on neuronal survival in the brain have previously been reported, GDNF effects on injured axons of the adult spinal cord have not been investigated. Using an ex vivo gene delivery approach that provides both trophic support and a cellular substrate for axonal growth, we implanted primary fibroblasts genetically modified to secrete GDNF into complete and partial mid‐thoracic spinal cord transection sites. Compared to recipients of control grafts expressing a reporter gene, GDNF‐expressing grafts promoted significant regeneration of several spinal systems, including dorsal column sensory, regionally projecting propriospinal, and local motor axons. Local GDNF expression also induced Schwann cell migration to the lesion site, leading to remyelination of regenerating axons. Thus, GDNF exerts tropic effects on adult spinal axons and Schwann cells that contribute to axon growth after injury. J. Comp. Neurol. 467:403–417, 2003. © 2003 Wiley‐Liss, Inc.
Morphological analysis of the hyperpolarization‐activated cyclic nucleotide‐gated cation channel 1 (HCN1) immunoreactive bipolar cells in the rabbit retina
Authors: Kim In‐Beom , Lee Eun‐Jin , Kang Tae‐Hoon , Chung Jin‐Woong , Chun Myung‐Hoon
Published: November 2003

Hyperpolarization‐activated cation currents (Ih) have been identified in neurons in the central nervous system, including the retina. There is growing evidence that these currents, mediated by the hyperpolarization‐activated cyclic nucleotide‐gated cation channel (HCN), may play important roles in visual processing in the retina. This study was conducted to identify and characterize HCN1‐immunoreactive (IR) bipolar cells by immunocytochemistry, quantitative analysis, and electron microscopy. The HCN1‐IR bipolar cells were a subtype of OFF‐type cone bipolar cells and comprised 10% of the total number of cone bipolar cells. The axons of the HCN1‐IR cone bipolar cells ramified narrowly in the border of strata 1 and 2 of the inner plexiform layer (IPL). These cells formed a regular distribution, with a density of 1,825 cells/mm2 at a position 1 mm ventral to the visual streak, falling to 650 cells/mm2 in the ventral periphery. Double‐labeling experiments demonstrated that their axons stratified narrowly within and slightly proximal to the OFF‐starburst amacrine cell processes. In the IPL, they were presynaptic to amacrine cell processes. The most frequent postsynaptic dyads formed of HCN1‐IR bipolar cell axon terminals are pairs composed of both amacrine cell processes. These results suggest that these HCN1‐IR cone bipolar cells might be the same as the DAPI‐Ba1 bipolar population, and might therefore be involved in a direction‐selective mechanism, providing inputs to the OFF‐starburst amacrine cells and/or the OFF‐plexus of the ON–OFF ganglion cells. J. Comp. Neurol. 467:389–402, 2003. © 2003 Wiley‐Liss, Inc.
The growth and photorefractive effect of co‐doped Ce:Cu:BSO crystals
Authors: Zheng Wei , Zhang Naidong , Zhao Liancheng , Xu Yuheng
Published: November 2003

In the paper Ce:Cu:BSO crystal has been grown by Czochralski method for the first time with doping CeO2 and CuO into BSO(Bi12SiO20) crystal. The exponential gain coefficient and respond time of Ce:Cu:BSO crystal are measured by two‐wave‐coupling technology. The results indicate that exponential gain coefficient of Ce:Cu:BSO is more than two times as that of non‐doped BSO and the response time exhibits in microsecond level. Furthermore its exponential gain coefficient improves greatly compared with Ce:BSO's at the same doping level of Ce, while its response time is less than Ce:BSO's. The improvement mechanism of photorefractive effect of Ce:Cu:BSO crystal is investigated systematically. (© 2003 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Preparation of (KBr‐KCl)(OH−):(F2+)H color center laser crystal series and their spectral characteristics
Authors: Wu Jihuai , Huang Changcang , Xu Chenghuang
Published: November 2003

In order to adjust and broaden the absorption spectrum and emission spectrum of the (F2+)H type color center so as to match up to the pumping and to fit in with the demand of application, a (KBr‐KCl)(OH−):(F2+)H color center laser crystal series was prepared by the process of single crystal growth, additive coloration, anneal, and light aggregation at 290 K and 77 K. The spectral characteristics of (KBr‐KCl)(OH−) crystals are investigated. It is found that the absorption and emission band of the (F2+)H color centers in KBr‐KCl complex host crystals has a considerable adjustment and extensions compared to the color centers in single KBr or KCl crystal. (© 2003 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Internet advertising: Is anybody watching?
Authors: Drèze Xavier , Hussherr François-Xavier
Published: November 2003

Click-through rates are still the de facto measure of Internet advertising effectiveness. Unfortunately, click-through rates have plummeted. This decline prompts two critical questions: (1) Why do banner ads seem to be ineffective and (2) what can advertisers do to improve their effectiveness? To address these questions, we utilized an eye-tracking device to investigate online surfers’ attention to online advertising. Then we conducted a large-scale survey of Internet users’ recall, recognition, and awareness of banner advertising. Our research suggests that the reason why click-through rates are low is that surfers actually avoid looking at banner ads during their online activities. This implies that the larger part of a surfer's processing of banners will probably be done at the pre-attentive level. If such is the case, click-through rate is an ineffective measure of banner ad performance. Our research also shows that banner ads do have an impact on traditional memory-based measure of effectiveness. Thus, we claim that advertisers should rely more on traditional brand equity measures such as brand awareness and advertising recall. Using such measures, we show that repetition affects unaided advertising recall, brand recognition, and brand awareness and that a banner's message influences both aided advertising recall and brand recognition.
At the movies, on the Web: An investigation of the effects of entertaining and interactive Web content on site and brand evaluations
Authors: Raney Arthur A. , Arpan Laura M. , Pashupati Kartik , Brill Dale A.
Published: November 2003

An experiment was conducted to examine the effects of including entertaining and/or interactive content on evaluations of automotive Web sites and automotive brands. Research participants visited and evaluated four Web sites from major automotive manufactures. The four sites exhibited varying degrees of entertaining content and interactivity. The highly entertaining site (which included a mini, suspenseful movie) was associated with the most positive site evaluations, greatest intent to return to the site, and highest levels of arousal, as compared to the three other sites that included video product footage only, video footage and audio, or video footage and audio with an interactive feature. Significant increases in purchase intent were associated only with the brand featured in the site with the mini movie.
A belief-updating process for minimizing waiting time in multiple waiting-time events: Application in website design
Authors: Weinberg Bruce D. , Berger Paul D. , Hanna Richard C.
Published: November 2003

Tim Berners-Lee, the inventor of the World Wide Web, has identified the waiting time problem, commonly referred to as the World Wide Wait, as a major problem that must be resolved in order for people to have rich robust Internet experiences. For the foreseeable future, slow download speeds may cost Web sites billions of dollars in opportunity loss. Using consumer behavior theory, we formulate a method for minimizing perceived waiting time in phenomena which involve multiple waits or time delays, here applied to Web site design and the Internet waiting problem. Solutions for two types of design situations are provided: (1) the optimal ordering of a predefined number of Web pages, and (2) an optimal number of Web pages and the amount of information on each page, for a set of information in a predefined order. We provide a solution for the general case, and a simplified solution for specific belief-updating patterns suggested by previous literature as frequently occurring in practice. This research contributes to the extant waiting time literature as well as provides solutions to, enhances understanding of, and encourages future research for, a persistent and important Internet and e-commerce problem that has received relatively little attention in the marketing literature.
Tell me no lies: Using science to connect with consumers
Authors: Hill Dan
Published: November 2003

Traditionally, we have viewed purchasing as a fundamentally rational process and have sought to persuade customers using reason-based features, attributes, and benefits marketing strategies. Market research testing has followed a parallel path, seeking logical verbal explanations to analyze consumers’ motivations. However, major findings in brain research over the last decade have altered our understanding of how we, as consumers, make decisions—suggesting that our most immediate “gut” reactions take place in the emotion center of the brain rather than in the rational neocortex. This article explores the key science concepts that are guiding us toward an approach to marketing that has more heart, that is more mind–body centered than cognitive. Specific research tools will be introduced along with strategic, psychological perspectives on direct-mail and Internet marketing.
Cover Picture: Recent Advances in the Chemistry of Metallacarboranes (Eur. J. Inorg. Chem. 22/2003)
Authors: Hosmane Narayan S. , Maguire John A.
Published: November 2003

The cover picture shows a typical bent‐sandwich commo‐metallacorborane product as well as its starting nido‐ycarborane precursor of the smaller carborane cage system, together in a novel cluster. Details are discussed in the Microreview by N. S. Hosmane and J. A. Maguire on p. 3989 ff. The authors are indebted to their many students and co‐workers who have borne the brunt of doing, and to the National Science Foundation, Alexander von Humboldt‐Stiftung (to N. S. H.) and the R. A. Welch Foundation (to J. A. M.) for their generous support.
The Reductive Fragmentation of 7‐Hydroxy‐9,10‐dioxotaxoids
Authors: Appendino Giovanni , Noncovich Alain , Bettoni Piergiorgio , Dambruoso Paolo , Sterner Olov , Fontana Gabriele , Bombardelli Ezio
Published: November 2003

The retro‐aldol reductive fragmentation of different structural types of 7‐hydroxy‐9,10‐dioxotaxoids was investigated, showing that the reaction is typical of taxanes and requires cerium(III) promotion with NaBH4 in protic medium and alkylboron (aluminium) hydrides in aprotic solvents. The resulting 7,8‐seco‐taxanes are key intermediates for the synthesis of a novel class of anticancer taxanes endowed with a unique pattern of in vivo biological activity. (© Wiley‐VCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2003)
A planar microfabricated nanoelectrospray emitter tip based on a capillary slot
Authors: Le Gac Séverine , Arscott Steve , Rolando Christian
Published: November 2003

We report on the fabrication and testing of planar nib‐like structures for nanoelectrospray ionization‐mass spectrometry (nanoESI‐MS) applications. The micro‐nib structures were fabricated on silicon substrates using the negative photoresist SU‐8; they include capillary slots with widths of 8 and 16 μm. A suitable wafer cleaving step made the nib‐like structures overhang the edge of a silicon substrate to provide a robust interface for nanoESI‐MS applications; this freeing of the nib tip from the wafer surface created a point‐like structure that is essential to establish an electrospray. The micro‐nib sources were successfully tested on an LCQ Deca XP+ ion trap mass spectrometer using peptide samples at concentrations down to 1 μM. The high voltage was applied using a platinum wire inserted in the sample reservoir upstream to the capillary slot. A Taylor cone was clearly seen at the nib tip. The micro‐nibs performed well at voltages as low as 0.8 kV; such performances are state‐of‐the‐art with respect to current micromachined ESI‐MS interfaces and are conditions comparable to those used for standard emitter tips. In addition, we clearly observed the influence of the micro‐nib slot width on the ionization performances: the narrower the slot, the better the performances.
Bdelloid Rotifers from Lakes above 1700 m in Western Italian Alps, with Taxonomic Notes on Dissotrocha macrostyla
Authors: Fontaneto Diego , Melone Giulio
Published: November 2003

Benthic and periphytic bdelloid communities from 16 alpine lakes from 1700 to 2850 m above sea level in Sesia Valley (Piedmont region, North‐western Italy), sampled during summer 2001 and 2002, were analyzed. Seventeen species were identified from these species‐poor communities, with 1 to 6 species each. Dissotrocha macrostyla and Philodina citrina were the most common species, present in 10 lakes while 9 species were collected from one lake only. New morphological details from S.E.M. pictures of Dissotrocha macrostyla revealed that Dissotrocha macrostyla tuberculata (Gosse, 1886) is only a seasonal morphotype. Its different appearance is due to the presence of locally distributed microscopic mucous bubbles (diameter 1.41 ± 0.18 μm) on the trunk surface, produced by the rotifer itself under stressful conditions.
The study of the sterilization effect of gamma ray irradiation of immobilized collagen polypropylene nonwoven fabric surfaces
Authors: Tyan Yu‐Chang , Liao Jiunn‐Der , Lin Shu‐Ping , Chen Chia‐Chieh
Published: November 2003

Exposure to gamma ray irradiation is a frequent, clean, and superior method used to prevent bacterial contamination of sterilized biomedical end products. However, the potential damage induced by gamma ray irradiation of collagen is of concern because of the decay of bioactivity, which correlates with considerable structural alterations. In this experiment, antenna‐coupling microwave plasma was utilized to activate nonwoven polypropylene (PP) fabric, and then the sample was grafted to acrylic acid (AAc). Type III collagen was immobilized by using water‐soluble 1‐ethyl‐3‐(3‐dimethylaminopropyl) carbodiimide as a coupling agent. The collagen‐immobilized samples, with temperatures of under 4°C, were exposed to gamma ray irradiation at different dose intervals. Gamma ray irradiation was applied to evaluate the bioactivity on the collagen‐immobilized nonwoven polypropylene and to determine the results of sterilization. Five kinds of sterilization index bacteria, all subject to Good Manufacturing Practice (GMP) criteria, were applied as a standard plate‐count sterilization test. Our experimental results demonstrate that in human plasma incubated with various intervals of gamma ray irradiation, fibrinogen concentration decreases while platelet and red blood cell adhesion increase. However, the dose required for thrombination demonstrated a significant change in gamma ray irradiation exposure of fewer than 10 KGy (p = 0.05). The decay of bioactivity of the gamma‐ray‐irradiated collagen‐bonded surfaces was evaluated and indicated that the decrease of R‐CONHR′, the degradation of amides (broken CN bonds of collagen and formation of the ROCNH2 and OCR′ bonds), and the increase of CO, CO bonds gradually may damage collagen by increasing the intervals of gamma ray irradiation. These effects considerably influence the bioactivity of the collagen‐bonded fabric. It is clear that gamma ray irradiation exposure of ≈10 KGy has the potential of moderating the bioactivities of collagen and therefore likely is a vital factor in the acceleration of biodegradation. The dose required for thrombination and sterilization reaches significance at 7.5 KGy. © 2003 Wiley Periodicals, Inc. J Biomed Mater Res 67A: 1033–1043, 2003
Culture of primary rat hepatocytes within porous chitosan scaffolds
Authors: Li Jieliang , Pan Jilun , Zhang Liguo , Guo Xinji , Yu Yaoting
Published: November 2003

Chitosan is considered to be a very promising biopolymer for various biomedical and pharmaceutical uses because of its nontoxic and biocompatible natures (Chandy T, Shama P. Biomater Artif Cells Artif Org 1990;18:1–24). In this study, we prepared porous chitosan scaffolds by lyophilization of chitosan solution. The scaffolds were modified with water‐soluble polyanionic species such as alginate and heparin. The pore structures of these scaffolds were viewed via light and scanning electron microscopy. The scaffolds prepared have a high porosity of ∼90% with mean pore sizes from 50 to 200 μm. They were used as substrates for hepatocytes culture. The cell attachment ratio was much higher than on monolayer membrane and hepatocytes exhibited a round cellular morphology with many microvilli evident on the surface of the cells. Metabolic activities of the cells were evaluated in terms of albumin secretion and urea synthesis. It was found that hepatocytes cultured on the modified scaffolds showed an increase in albumin secretion during the first 4 days and were more stable than those on monolayer membrane and nonmodified scaffolds. Therefore, primary rat hepatocytes cultured on modified scaffolds would be beneficial to liver assist device. © 2003 Wiley Periodicals, Inc. J Biomed Mater Res 67A: 938–943, 2003
Public–private community partnerships in infrastructure for the poor
Authors: Franceys Richard , Weitz Almud
Published: November 2003

There is an urgent need to improve the delivery of water and environmental sanitation services to the urban poor in low and middle‐income countries to protect health and to promote sustainable livelihoods. Conventional support to public providers through loans for capital investment linked to capacity building has not achieved the desired results. There is a growing awareness of the need for institutional reform to overcome the all too common failures if service to the poor is to reach an acceptable level.

The urban poor in Asia typically represent one quarter to one half of a fast growing urban area's population often dwelling in unplanned areas without any security of tenure. A study was undertaken in ten Asian countries to investigate the roles of and interaction between the public sector, private entities and civil society in serving the urban poor with water supply, sanitation and solid waste management. Case studies, including focus group discussions with low‐income slum residents, were undertaken by country‐based researchers distributed over a spectrum of service type, country population, city size and institutional/contract type to investigate the benefits of infrastructure partnerships.

Analysis of the 20 studies confirmed the limited ability of existing public entities to reach the poor. The essential need for some level of service provision was being met by a mixture of poor quality self‐provision, by informal private entities often at high, unregulated prices, or by innovative civil society involvement experiencing difficulties in scaling‐up. A number of the new public private approaches were found to be successfully reforming the direct public providers; however these were usually serving poor people only by default through the general improvement in performance. A very limited number of public private community partnerships with international operators were found to be dramatically improving service to some of the poor, with better quality at lower price, but often with long‐term uncertainty over contractual stability. Copyright © 2003 John Wiley & Sons, Ltd.