bluuebunny/crossref_metadata_2025_split huggingface dataset:
Riluzole attenuates spinal muscular atrophy disease progression in a mouse model
Authors: Haddad Hafedh , Cifuentes‐Diaz Carmen , Miroglio Audrey , Roblot Natacha , Joshi Vandana , Melki Judith 
Published: September 2003

AbstractSpinal muscular atrophy (SMA) is a motor neuron disease caused by mutations of the survival motor neuron 1 gene (SMN1). No curative treatment is available. Mutant mice carrying homozygous deletion of Smn exon 7 directed to neurons display a degenerative process of motor neurons similar to that found in human SMA. To test whether riluzole, which exhibits neurotrophic properties, might have a protective role in SMA, mutant mice were treated with it after the onset of the degenerative process. Riluzole improved median survival and exerted a protective effect against aberrant cytoskeletal organization of motor synaptic terminals but not against loss of proximal axons. These results demonstrate that the disease course of SMA can be attenuated after the onset of neuromuscular defects and may warrant further investigation in a therapeutic trial in SMA. Muscle Nerve 28: 432–437, 2003
Late‐onset distal muscular dystrophy affecting the posterior calves
Authors: Katz Jonathan S. , Rando Thomas A. , Barohn Richard J. , Saperstein David S. , Jackson Carlayne E. , Wicklund Matthew , Amato Anthony A. 
Published: September 2003

AbstractMiyoshi myopathy, caused by mutations in the membrane protein dysferlin, is the most common muscular dystrophy that presents in the posterior calves. Its onset is before the age of 30 years and it is associated with marked elevations of serum creatine kinase (CK). In contrast, little is known about calf myopathies with onset after the age of 30, and it is not clear whether such patients have a dysferlinopathy. We describe five patients with a myopathy predominantly affecting the calf muscles, with onset after the age of 30. Muscle tissue was analyzed by immunoblot for dystrophin and dysferlin. All five had normal dysferlin but one had a dystrophinopathy. Serum CK levels ranged from 3 to 15 times the upper limit of normal. In contrast, all of 13 patients presenting before age 30 with calf weakness had a dysferlinopathy. Thus, isolated calf atrophy and weakness with onset after age 30, and associated with serum CK levels that are only moderately elevated, represents a distinct myopathy phenotype. Most of these cases are sporadic, although the overall phenotype appears genetically heterogeneous and dysferlinopathy is uncommon. Muscle Nerve 28: 443–448, 2003
Increased jitter and blocking in normal muscles due to doubly innervated muscle fibers
Authors: Lateva Zoia C. , McGill Kevin C. , Johanson M. Elise 
Published: September 2003

AbstractIncreased jitter and intermittent impulse blocking in electromyographic (EMG) signals are considered evidence of transmission abnormality and are not usually associated with normal muscle. However, motor unit action potentials (MUAPs) that exhibit increased jitter and blocking have recently been shown to occur in the brachioradialis muscles of neurologically healthy subjects. The jitter and blocking result from collisions, refractoriness, and conduction‐velocity variability in long muscle fibers that are innervated by two different motoneurons at widely separated endplates. We analyzed MUAPs obtained by decomposing EMG signals from the brachioradialis muscles of four normal subjects. The rate of blocking of some MUAP components was as high as 28%, the jitter between some components exceeded 300 μs (mean consecutive difference), and the mean incidence of irregular MUAPs was 14%. These values would be considered abnormal in many other muscles. Jitter from doubly innervated fibers can be distinguished from other types of pathological jitter because one component exhibits amplitude variability. Clinical neurophysiologists should be aware that increased jitter and blocking do not necessarily indicate pathology in brachioradialis and perhaps other long, parallel‐fibered muscles. Muscle Nerve 28: 423–431, 2003
Near‐nerve potential in lepromatous leprosy
Authors: Marques Wilson , Foss Norma T. , Arruda Ana P. M. , Barreira Amilton A. 
Published: September 2003

AbstractIn leprosy, sensory action potentials (SAPs) may be normal in spite of clinical sensory loss. This may result from the early involvement of small nerve fibers, which have potentials that are not detected in routine studies. To evaluate this possibility, we used a near‐nerve recording technique that records potentials from nerve fibers as small as 4–6 μm in diameter. We hypothesized that this technique might increase the sensitivity of nerve conduction studies in detecting leprosy neuropathy. We found the technique to be useful for recording conduction abnormalities in recently diagnosed patients, including those with preserved sensation, suggesting that axonal loss may be the underlying mechanism. Contrary to our hypothesis, however, recording the late SAP components did not improve the sensitivity of nerve conduction studies. We suggest that the late components having normal conduction velocities may be generated by either regenerating or remyelinating abnormal fibers, which have an electrophysiological behavior similar to that of normal 4–6‐μm‐diameter fibers. Muscle Nerve 28: 460–463, 2003
Calpain 3 cleaves filamin C and regulates its ability to interact with γ‐ and δ‐sarcoglycans
Authors: Guyon Jeffrey R. , Kudryashova Elena , Potts Alexandra , Dalkilic Isin , Brosius Melissa A. , Thompson Terri G. , Beckmann Jacques S. , Kunkel Louis M. , Spencer Melissa J. 
Published: September 2003

AbstractCalpain 3 (C3) is the only muscle‐specific member of the calcium‐dependent protease family. Although neither its physiological function nor its in vivo substrates are known, C3 must be an important protein for normal muscle function as mutations in the C3 gene result in limb‐girdle muscular dystrophy type 2A. Previous reports have shown that the ubiquitous calpains (μ and m) proteolyze filamins in nonmuscle cells. This observation suggests that the muscle‐specific filamin C (FLNC) is a good candidate substrate for C3. Binding studies using recombinant proteins establish that recombinant C3 and native FLNC can interact. When these two proteins are translated in vitro and incubated together, C3 cleaves the C‐terminal portion of FLNC. Cleavage is specific as C3 fails to cleave FLNC lacking its C‐terminal hinge and putative dimerization domains. Cotransfection experiments in COS‐7 cells confirm that C3 can cleave the C‐terminus of FLNC in live cells. The C‐terminus of FLNC has been shown to bind the cytoplasmic domains of both δ‐ and γ‐sarcoglycan. Removal of the last 127 amino acids from FLNC, a protein that mimics FLNC after C3 cleavage, abolishes this interaction with the sarcoglycans. These studies confirm that C3 can cleave FLNC in vitro and suggest that FLNC may be an in vivo substrate for C3, functioning to regulate protein–protein interactions with the sarcoglycans. Thus, calpain‐mediated remodeling of cytoskeletal–membrane interactions, such as those that occur during myoblast fusion and muscle repair, may involve regulation of FLNC–sarcoglycan interactions. Muscle Nerve 28: 472–483, 2003
Overexpressions of myoglobin and antioxidant enzymes in ragged‐red fibers of skeletal muscle from patients with mitochondrial encephalomyopathy
Authors: Kunishige Makoto , Mitsui Takao , Akaike Masashi , Kawajiri Masakazu , Shono Masayuki , Kawai Hisaomi , Matsumoto Toshio 
Published: September 2003

AbstractTo determine the relationship between myoglobin (Mb) and the defense system against reactive oxygen species in various myopathies, we performed immunohistochemical analyses of Mb and various antioxidant enzymes, including manganese superoxide dismutase (Mn‐SOD), copper zinc SOD (CuZn‐SOD), catalase (CAT), and glutathione peroxidase (GSH‐Px). Biopsied muscle specimens were obtained from patients with chronic progressive external ophthalmoplegia (CPEO), Kearns–Sayre syndrome (KSS), Duchenne muscular dystrophy (DMD), and polymyositis (PM). In patients with CPEO/KSS, stainings of Mb, SOD, CAT, and GSH‐Px in nonatrophic ragged‐red fibers (RRFs) were more intense than those in non‐RRFs. These pronounced stainings corresponded to ragged‐red lesions. The staining intensities of these antioxidant enzymes were significantly correlated with that of Mb (P < 0.001). Atrophic RRFs in specimens from patients with CPEO/KSS showed intense stainings of these antioxidant enzymes but not intense staining of Mb. In specimens from patients with DMD/PM, the antioxidant enzymes but not Mb were overexpressed in degenerative fibers. These results suggest that oxidative stress is associated with Mb expression specifically in mitochondrial diseases. The antioxidant enzymes seem to be upregulated to protect against muscle damage in nonatrophic RRFs. However, the Mb‐mediated oxidative damage may become more extensive and result in further mitochondrial dysfunction and progressive atrophy of RRF with impaired upregulation of Mb. Muscle Nerve 28: 484–492, 2003
Mechanisms of force loss in diabetic mouse skeletal muscle
Authors: Lesniewski Lisa A. , Miller Todd A. , Armstrong R. B. 
Published: September 2003

AbstractPathologic changes to α‐motoneurons may contribute to decreases in skeletal muscle strength in diabetes. The present study examines this possibility. Female ICR mice (∼25 g) were given a single injection of streptozotocin (200 mg/kg). After 2, 4, and 8 weeks of diabetes, we measured maximum isometric tetanic torque of the fast‐twitch anterior crural muscles at the ankle when stimulated through the common peroneal nerve, and maximal isometric tetanic force in the directly stimulated extensor digitorum longus (EDL) muscle. After 4 weeks, the relative loss of torque via nerve stimulation (−43%) was greater (P = 0.02) than the force loss in the directly stimulated muscle (−24%), indicating a functional neural deficit. However, the percent changes in strength in these two methods of stimulation were not different (P = 0.41) in the 8‐week diabetic animals, indicating that functional impairment resided in the muscle. This suggests an early distal motoneuron or neuromuscular junction deficit that improved as the intrinsic muscle deficit worsened. Preliminary evidence also suggests excitation–contraction uncoupling may contribute to the loss of strength in fast‐twitch muscles. Muscle Nerve 28: 493–500, 2003
Changes in the mechanical properties of tooth‐colored direct restorative materials in relation to time
Authors: Saygılı Gülbin , Şahmalı Sevil , Demirel Figen 
Published: September 2003

AbstractThe objective of this study was to determine the flexural strength, flexural modulus, Vickers hardness of a packable composite (Surefil), and an ormocer (Definite) in comparison with a microhybrid composite (Z‐100), a microfil composite (Silux Plus) and a polyacid‐modified composite resin (Dyract). Flexural strength and flexural modulus were determined using a three‐point bending device. Microhardness was measured with a Vickers indentor. The specimens of each material were prepared according to manufacturer's instructions.The specimens were stored in artificial saliva at pH 6, all at 37°C. The groups were tested at the beginning of the test, at 3 months and at 6 months. Flexural strength values of Surefil and Definite showed a progressive increase. The highest MPa values were determined for Surefil (134.4 MPa) and the lowest MPa values were obtained for Dyract (59.6 MPa). The highest flexural modulus values were revealed for Surefil (10.000 GPa). Z‐100, Silux Plus and Definite showed a tendency to decline in relation to time for their flexural modulus. GPa values of Silux Plus were stable at 3 and 6 months. Vickers hardness numbers showed that Surefil was the hardest and Dyract was the weakest material. Copyright © 2003 John Wiley & Sons, Ltd.
Predictability of combined effects of eight chloroacetanilide herbicides on algal reproduction
Authors: Junghans Marion , Backhaus Thomas , Faust Michael , Scholze Martin , Grimme L Horst 
Published: September 2003

AbstractChloroacetanilides are pre‐emergence herbicides for the control of annual grasses and broad‐leaf weeds. As a result of their extensive use, residues are often found in surface waters. Observed simultaneous occurrence of different chloroacetanilide herbicides gives reason for concern about potential combination effects on aquatic non‐target organisms. This study aimed to clarify whether joint effects of various chloroacetanilide herbicides may be predictable from knowledge of concentration–response relationships of single substances. Whether the chloroacetanilides all share the same mode of action is unclear. Therefore we investigated the predictive value of two alternative concepts for the prediction of combined effects: concentration addition, which assumes a similar mode of action, and independent action, which is based on the idea of a dissimilar mode of action of the mixture components. Eight chloroacetanilides (acetochlor, alachlor, butachlor, dimethachlor, metazachlor, metolachlor, pretilachlor and propachlor) were experimentally tested for their individual as well as for their combined effects in mixtures on the reproduction of the green algaScenedesmus vacuolatus. Individual chloroacetanilides impaired algal reproduction, with EC50values ranging from 3 to 232 µg litre−1. The differences in EC50values were strongly correlated with the lipophilicities of the compounds. Effects of chloroacetanilide mixtures were considerably higher than those of the individual components: a complete inhibition of algal reproduction was observed when every mixture component was present in a concentration that would cause only 5% effect if applied singly. However, the combined effects proved to be predictable by using the concept of concentration addition. The alternative concept, independent action, distinctly underestimated the mixture toxicity. These findings (1) indicate a similar mechanism of action of chloroacetanilides in algae and (2) reinforce the view that concentration addition is a reasonable assumption for the predictive hazard assessment of groups of similarly acting herbicides. Copyright © 2003 Society of Chemical Industry
The Hare and the Tortoise Revisited: The New Politics of Consumer and Environmental Regulation in Europe
Authors: VOGEL DAVID 
Published: September 2003

There has been an important shift in the pattern of divergence between consumer and environmental protection policies in Europe and the United States. From the 1960s through the mid 1980s American regulatory standards tended to be more stringent, comprehensive and innovative than in either individual European countries or in the European Union (EU). However, since around 1990 the obverse has been true; many important EU consumer and environmental regulations are now more precautionary than their American counterparts.The ‘new’ politics of consumer and environmental regulation in Europe are attributable to three inter-related factors: a series of regulatory failures within Europe, broader and stronger political support for more stringent and comprehensive regulatory standards within Europe and the growth in the regulatory competence of the European Union.In many respects, European regulatory politics and policies since the 1990s resemble those of the United States during the 1970s. Thus health, safety and environmental politics and policies in the United States are no longer as distinctive as many scholars have portrayed them.
IgE‐binding epitopes of the American cockroach Per a 3 allergen
Authors: Wu C. H. , Lee M. F. , Tseng C. Y. 
Published: September 2003

Background: The Per a 3 is a species‐specific allergen of the American cockroach (Periplaneta americana) related to insect hemolymph proteins and includes four known isoallergens. This study aimed to identify Per a 3 linear IgE‐binding epitopes.Methods: Per a 3 recombinant fragments were generated from the recombinant Per a 3.01 allergen (685 amino acid residues) by using existing restriction sites or by using polymerase chain reaction products, and expressed in Escherichia coli. Antigenicities were assessed by immunoblotting, enzyme‐linked immunosorbent assay (ELISA), and binding inhibition with human IgE.Results: Human IgE recognized recombinant fragments 340–425, 466–579, 502–595, and 595–636 as revealed by immunoblotting and ELISA. On the other hand, the N‐terminal fragment 1–399, recombinants 410–443, 472–551, 502–579, 606–636, and the C‐terminal fragment 636–685 were unable to bind human IgE. Amino acid sequences 400–409, 466–471, 580–595, and 595–605 were shown to be required for IgE binding to the Per a 3.01 allergen, suggesting that the C‐terminus contains most of the IgE‐binding sites. Four peptides corresponding to these IgE‐binding amino acid sequences were synthesized. These peptides reacted with most sera (62.5–87.5%) tested as revealed by ELISA, demonstrating a heterogeneous IgE‐binding response. Moreover, preincubation of IgE‐positive recombinant proteins and synthetic peptides with atopic IgE resulted in marked inhibition of the IgE binding to Per a 3.01 allergen. Amino acid sequences 400TVLRDPVFYQ409, 466NNVDQI471, 580VDKGHNYCGYPENLLI595, and 595IPKGKKGGQAY605 of the major recombinant American cockroach Per a 3.01 allergen were involved in IgE binding.Conclusion: These findings will advance our understanding of the antigenic structures responsible for allergenicity to the American cockroach, thereby providing strategies for the development of immunotherapies.
Laboratory assessment of the efficiency of encasing materials against house dust mites and their allergens
Authors: Mahakittikun V. , Komoltri C. , Nochot H. , Angus A. C. , Chew F. T. 
Published: September 2003

Background: The current recommendation to reduce mite allergen exposure for mite‐sensitive individuals is to use allergen‐impermeable bed coverings. As these covers are made of various kinds of materials, they vary in quality. The objective of this study was to investigate the efficiency of different covering materials against house dust mites and their allergens in vitro.Methods: Four types of materials including (1) plastic cover, (2) polyurethane‐coated cover, (3) non‐woven covers, (4) tightly woven microfiber covers and a regular cotton bed sheet (as a control) were evaluated using three methods: (i) heat escape method, (ii) Siriraj chamber method and stereomicroscopy, scanning electron microscopy and (iii) enzyme‐linked immunosorbent assay (ELISA).Results: We found that there was a statistically significant difference in allergen permeability among four types of coverings (P < 0.001). In terms of the impermeability to mites and their allergens, plastic‐ and polyurethane‐coated covers were observed to be the best, followed by non‐woven, woven and cotton‐based bed sheets. A regular cotton‐based bed sheet allows a significant amount of leakage of mite allergens. Both woven and non‐woven material are efficient barriers against mite allergen in terms of impermeability. However, with regard to mite colonization, non‐woven covers have the drawback of mites being able to penetrate and colonize within the fabric fibers. Woven covers are therefore recommended because of their major advantages of not allowing the colonization of mites within the fabric, being easy to clean, and comfortable.Conclusion: The three assessment methods used in this study could be useful as a primary approach to evaluate the quality of covering materials in vitro using both pore size and ability to be colonized by mites on the materials as the key factors.
Molecular and immunological characterization of Pen ch 18, the vacuolar serine protease major allergen of Penicillium chrysogenum
Authors: Shen H.‐D. , Chou H. , Tam M. F. , Chang C.‐Y. , Lai H.‐Y. , Wang S.‐R. 
Published: September 2003

Background: We have suggested previously that the 32 and 34 kDa major allergens of Penicillium chrysogenum (also known as P. notatum) are the vacuolar (Pen ch 18) and the alkaline (Pen ch 13) serine proteases, respectively, of P. chrysogenum. The purpose of this study is to characterize the 32 kDa allergen of P. chrysogenum and its immunoglobulin E (IgE)cross‐reactivity with Pen ch 13 allergen.Methods: The full‐length cDNA of Pen ch 18 was isolated by reverse transcriptase‐polymerase chain reaction and the 5′‐rapid amplification cDNA end reaction. Recombinant Pen ch 18 was expressed as his‐tagged proteins in Escherichia coli. Its reactivity with IgE and monoclonal antibodies against fungal serine protease allergens was analyzed by immunoblotting. The IgE cross‐reactivity between Pen ch 18 and Pen ch 13 was analyzed by immunoblot inhibition. Overlapping recombinant fragments and synthetic peptides were used to map the B cell epitopes on Pen ch 18.Results: In this study, we isolated a 1857 bp cDNA fragment containing an open reading frame of 494 amino acids that encodes the preproenzyme of Pen ch 18. Similar to other vacuolar serine proteases, this precursor appears to undergo N‐ and possibly C‐terminal cleavage upon maturation. The his‐tagged recombinant Pen ch 18 containing the putative sequence of the mature protein reacted with IgE antibodies in serum samples from asthmatic patients. In addition, IgE‐binding to the 32 kDa major allergen of P. chrysogenum was inhibited when a positive serum sample was absorbed with recombinant Pen ch 18 before immunoblotting. Both inhibition and almost no inhibition of IgE‐binding to the 32 kDa major allergen of Pen ch 18 were detected when eight positive serum samples were preabsorbed individually with purified Pen ch 13 before immunoblotting. The major IgE binding region was located in a fragment (PN1) encompassing the N‐terminal 102 amino acid residues of the recombinant Pen ch 18. A dominant linear IgE epitope was further mapped within residues 73–95 (peptide PN1‐e) of the N‐terminally processed allergen. Monoclonal antibody FUM20 that reacts with Pen ch 18 but not with Pen ch 13 binds a synthetic peptide with sequence encompassing the N‐terminal 23 residues of the recombinant Pen ch 18. Monoclonal antibody PCM39 that reacts with both Pen ch 13 and Pen ch 18 recognizes a peptide containing residues 132–154 of the allergen.Conclusions: Our results confirm that the Pen ch 18 allergen is a vacuolar serine protease of P. chrysogenum that matures through N‐ and possibly C‐terminal processing. The finding that there are cross‐reactive and allergen‐specific IgE epitopes for Pen ch 18 and Pen ch 13 suggests that both major allergens should be included in clinically diagnostic P. chrysogenum extracts.
Allergenicity of recombinant Bla g 7, German cockroach tropomyosin
Authors: Jeong K. Y. , Lee J. , Lee I.‐Y. , Ree H.‐I. , Hong C.‐S. , Yong T.‐S. 
Published: September 2003

Background:Cockroach infestation may sensitize and elicit allergic responses to genetically predisposed individuals. Invertebrate tropomyosins are a frequent cause of allergy and highly cross‐reactive in nature. In this study, we aimed to produce recombinant German cockroach tropomyosin and investigate its allergenicity.Methods:German cockroach tropomyosin (Bla g 7) was cloned by reverse transcriptase polymerase chain reaction (RT‐PCR). The cloned cDNA was over‐expressed inEscherichia coliand purified by affinity chromatography using Ni‐nitrilotriacetic (NTA) acid resin. The allergenicity of the recombinant tropomyosin was examined by enzyme‐linked immunosorbent assay (ELISA).Results:The cloned Bla g 7 shared up to 91% amino acid sequence identity with other cockroach tropomyosins. ELISA showed a recombinant Bla g 7 sensitization rate of 16.2% to German cockroach allergic sera. Recombinant tropomyosin was able to inhibit 32.4% of the specific IgE binding to cockroach extract.Conclusions:Tropomyosin represents a minor allergen in cockroach extracts. It is hoped that recombinant tropomyosin will be useful for further studies and clinical applications.