bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mutations in the amino terminus of ANKH in two US families with calcium pyrophosphate dihydrate crystal deposition disease
Authors: Williams Charlene J. , Pendleton Adrian , Bonavita Gina , Reginato Antonio J. , Hughes Anne E. , Peariso Shelly , Doherty Michael , McCarty Daniel J. , Ryan Lawrence M. 
Published: September 2003

AbstractObjectiveTo analyze ANKH in families with calcium pyrophosphate dihydrate crystal deposition disease (CPPD) for disease‐causing mutations.MethodsTwo US families (one of British ancestry and the other of German/Swiss ancestry) with autosomal‐dominant CPPD, whose disease phenotypes were found to be linked to chromosome 5p15.1 (locus symbol CCAL2), were screened by direct sequencing for mutations in ANKH, a gene in the CCAL2 candidate interval that has been shown to harbor mutations in other families with CPPD. Observed sequence variants were confirmed by antisense sequencing, and expression of the mutant allele was verified by reverse transcriptase–polymerase chain reaction amplification of messenger RNA followed by direct sequencing.ResultsThe two US families displayed the same mutation at position 5 of the ANKH gene product (P5T). All affected members were heterozygous for the P‐to‐T variant, and the mutation was not seen in 204 control alleles. The two families displayed distinct disease haplotypes, suggesting that they were unrelated to each other.ConclusionThese observations represent the fourth and fifth families with heritable CPPD whose disease phenotypes are linked to the CCAL2 locus and who have missense mutations in the amino terminus of ANKH. This same position (P5) was the site of a missense mutation in an Argentinean family of northern Italian ancestry; however, the sequence variant in that family generated a P5L mutation. The distinct disease haplotypes among the 3 families with P5 mutations suggest that the mutations arose independently and that the evolutionarily conserved P5 position of ANKH may represent a hot spot for mutation in families with autosomal‐dominant CPPD.
Enhanced degradation of proteins of the basal lamina and stroma by matrix metalloproteinases from the salivary glands of Sjögren's syndrome patients: Correlation with reduced structural integrity of acini and ducts
Authors: Goicovich Eduardo , Molina Claudio , Pérez Paola , Aguilera Sergio , Fernández Juan , Olea Nancy , Alliende Cecilia , Leyton Cecilia , Romo Rafael , Leyton Lisette , González María‐Julieta 
Published: September 2003

AbstractObjectiveTo determine the effect of matrix metalloproteinase (MMP) activity from the labial salivary glands (LSGs) of Sjögren's syndrome (SS) patients on proteins of the extracellular matrix (ECM) that form the basal lamina and stroma, and to compare this effect with the structural integrity of acini and ducts as well as the functionality of the LSGs.MethodsGelatinase activity was determined by zymography. The digestion pattern of extracellular matrix (ECM) macromolecules was detected by gel electrophoresis and quantified by densitometry. The structural integrity of acini and ducts was evaluated by light and electron microscopy. Secretory function was evaluated by measuring unstimulated salivary flow and by scintigraphy.ResultsLSG extracts showed increased levels of proteolytic activity toward purified proteins of the basal lamina (laminin and type IV collagen) and stroma (types I and III collagen and fibronectin). Enhanced degradation was most evident for fibronectin, laminin, and type IV collagen. Analysis of the ultrastructure of the acinar and ductal basal lamina revealed abnormalities ranging from disorganization to disappearance of this ECM structure. These changes were paralleled by an important loss of microvilli on the apical surface, as well as decreased unstimulated salivary flow. Interestingly, the results were similar in LSGs from all SS patients, regardless of the proximity of infiltrating mononuclear cell foci.ConclusionOur observation that the proteolytic action of MMPs toward ECM macromolecules is increased in SS patients provides a rationale for understanding the dramatic changes in the structural organization observed in the basal lamina and apical surface of acini in these patients. The results provide new evidence that acinar and ductal cells from the LSGs of SS patients display a molecular potential, with increased capacity to markedly disorganize their ECM environment and, thus, damage their architecture and functionality.
Distinct mechanisms of action of anti‐CD154 in early versus late treatment of murine lupus nephritis
Authors: Quezada Sergio A. , Eckert Maria , Adeyi Oyedele A. , Schned Alan R. , Noelle Randolph J. , Burns Christopher M. 
Published: September 2003

AbstractObjectiveTreatment with anti‐CD154 antibody is known to ameliorate murine lupus nephritis when given early in the disease. The aims of this study were to identify the mechanism of this early effect, to determine whether late anti‐CD154 treatment could halt established nephritis, and, if so, to examine potential mechanisms of late efficacy.MethodsWe studied the effects of anti‐CD154 treatment on autoantibody production and immune complex deposition, renal pathology, survival, and renal cytokine and chemokine messenger RNA (mRNA) expression both in (NZB × NZW)F1 mice (BW mice) and in NZM.2410 mice.ResultsEarly treatment with anti‐CD154 produced long‐term survival in BW mice, with abrogation of renal immune complex deposition for months after treatment was stopped. Late anti‐CD154 treatment, started after development of nephritis, could halt disease in ∼40% of mice. In some mice, proteinuria could be reversed repeatedly with sequential courses of anti‐CD154 antibody. The remissions induced by late treatment with anti‐CD154 occurred despite ongoing renal immune complex deposition. In preliminary studies, responding mice had rapid reductions in renal mRNA for transforming growth factor β, interleukin‐10, and tumor necrosis factor α.ConclusionAmelioration of murine lupus by anti‐CD154 therapy is mediated by distinct mechanisms in early versus late intervention. We postulate that anti‐CD154 therapy prevents autoantibody production and renal immune complex deposition in the early, induction phase and limits secondary tissue damage in situ in the late, effector phase. These data demonstrate that CD40–CD154 interactions are critical for the maintenance of autoimmunity and suggest a potential role for anti‐CD154 as a therapeutic agent in established human lupus.
Hospitalizations for coronary artery disease among patients with systemic lupus erythematosus
Authors: Thorburn Christine M. , Ward Michael M. 
Published: September 2003

AbstractObjectiveAlthough patients with systemic lupus erythematosus (SLE) have an increased risk of coronary artery disease (CAD) compared with persons without SLE, the burden of CAD among SLE patients is unknown. This study was undertaken to estimate this burden.MethodsWe used the Nationwide Inpatient Sample of the Healthcare Cost and Utilization Project to estimate the number of hospitalizations for CAD among patients with SLE in the US in 1998. CAD diagnoses included acute myocardial infarction (MI), unstable angina, cardiac catheterization, percutaneous transluminal coronary angioplasty, or coronary artery bypass grafting as the primary reason for hospitalization. We compared these estimates with the frequency of hospitalization for other reasons.ResultsThere were an estimated 98,217 hospitalizations among patients with SLE in 1998. Of these, 11,947 (12%) were among men, 43,674 (44%) were among women <50 years of age, and 42,596 (43%) were among women ≥50 years of age. There were 4,951 hospitalizations for CAD, with 1,763 of these for acute MI. In women <50 years old, there were an estimated 311 hospitalizations for MI. Hospitalizations for CAD were less common than hospitalizations for SLE itself or for infections, and in young women, were less common than hospitalizations for complications of chronic renal failure.ConclusionCAD is an important comorbid condition in patients with SLE, but is not as common a reason for hospitalization as SLE itself, infections, and, in some patient subgroups, chronic renal failure.
Activin A induces cell proliferation of fibroblast‐like synoviocytes in rheumatoid arthritis
Authors: Ota Fumie , Maeshima Akito , Yamashita Shin , Ikeuchi Hidekazu , Kaneko Yoriaki , Kuroiwa Takashi , Hiromura Keiju , Ueki Kazue , Kojima Itaru , Nojima Yoshihisa 
Published: September 2003

AbstractObjectiveTo investigate the expression of activin A and its receptors in rheumatoid arthritis (RA) synovial tissues, and to determine the effect of activin A on cultured fibroblast‐like synoviocytes (FLS).MethodsThe localization of activin A and activin type II receptor (ARII) in synovial tissues of RA patients was analyzed by immunohistochemistry. The expression of activin A and activin receptors in human cultured FLS was examined by reverse transcriptase–polymerase chain reaction and Western blotting. Enzyme‐linked immunosorbent assay was used to measure activin A in culture supernatants. The cell growth of FLS was determined by 3H‐thymidine incorporation and MTT assay.ResultsImmunohistochemical analysis confirmed the up‐regulation of activin A in rheumatoid synovium as compared with osteoarthritis or normal joint tissues. CD68+ macrophage‐lineage cells and vimentin‐positive FLS were identified as activin‐producing cells in rheumatoid synovium. Both cell types also expressed ARII. The expression of activin A and ARII on cultured FLS was confirmed at the protein and messenger RNA levels. Interleukin‐1β (IL‐1β), tumor necrosis factor α, and transforming growth factor β activated FLS to secrete activin A. Recombinant activin A accelerated the proliferation of FLS, while follistatin, an endogenous activin antagonist, partially inhibited FLS proliferation induced by IL‐1β.ConclusionThese results suggest that activin A acts as a growth factor of FLS in RA.
Criminal careers behind bars
Authors: DeLisi Matt 
Published: September 2003

AbstractIn the criminal career literature, prison is usually viewed as an interruption of offending. Little research has applied the criminal career perspective to misconduct committed by prisoners. The current study used official data from a probability sample of 1,005 inmates from the southwestern United States to examine offending careers during incarceration. Descriptive and negative binomial regression analyses produced findings that suggest both similarities and dissimilarities between conventional and prison criminal careers. The incidence and prevalence of crime were inversely related to its seriousness but the magnitude of crime was higher in prison. One‐third of inmates were never contacted for a prison violation and 74% were never contacted for a serious/violent violation. On the other hand, 40% of inmates were chronic or extreme career offenderseven while incarcerated. A small cadre of inmates accounted for 100% of the murders, 75% of the rapes, 80% of the arsons, and 50% of the aggravated assaults occurring behind bars. Finally, prior criminality was the best predictor of prison offending, which is supportive of the importation model. While a significant number of inmates fully comply with prison rules, an even larger percentage of inmates continue to commit an array of crimes and rule violations despite the efforts of prison officials. Copyright © 2003 John Wiley & Sons, Ltd.
Ethical and legal standards for research in prisons
Authors: Kalmbach K. C. , Lyons Phillip M. 
Published: September 2003

AbstractBiobehavioral research, especially that which is conducted with prisoners, has become much more closely regulated in the last 30 years. State and federal law, as well as professional standards, regulate the conduct of many types of research; in the case of prisoners, this regulation is even more stringent. However, currently no mandatory, uniform, national regulatory or oversight process exists, and many privately funded research endeavors are operating in a regulatory void. In response to this, the National Bioethics Advisory Commission has argued for the creation of a single, national, independent regulatory body to oversee all human participant research, regardless of funding source. As ethicolegal research standards evolve alongside advances in science and technology, an appreciation of the history of prisoner research and an awareness of current standards is critical to conducting ethical prison research. Copyright © 2003 John Wiley & Sons, Ltd.
Role of cytology in the diagnosis of Barrett's esophagus and associated neoplasia
Authors: Saad Reda S. , Mahood Laura K. , Clary Karen M. , Liu Yulin , Silverman Jan F. , Raab Stephen S. 
Published: September 2003

AbstractWe studied 327 consecutive paired esophageal biopsies and brushing specimens obtained during the same endoscopic session to evaluate the role of cytology for the diagnosis of Barrett's esophagus (BE) and/or surveillance for associated dysplasia. A diagnosis of BE was based on the cytologic presence of goblet cells. Cases were reviewed and categorized into: 1) benign esophageal lesions (125 cases), with 48 cases of Candida (32 cases diagnosed by both techniques and 16 diagnosed only by cytology), 3 cases of herpes simplex with only 1 case diagnosed by cytology, and 74 cases of inflammation and/or repair; 2) benign BE (141 cases), with 74 cases (52%) diagnosed by both techniques, 11 cases by cytology only (8%), and 56 cases (40%) by histology only; 3) low‐grade dysplasia (LGD, 30 cases), with 5 cases (17%) diagnosed with both specimens, one case (3%) by cytology only, and 24 cases (80%) by histology only; 4) high‐grade dysplasia (HGD, 10 cases), with 8 cases (80%) diagnosed with both specimens, 1 case (10%) by cytology, and 1 case (10%) by histology; and 5) carcinomas (23 cases), with 20 cases (87%) diagnosed with both specimens, 2 cases (9%) by cytology only, and 1 case (4%) by histology only. Our results support the high degree of diagnostic accuracy of cytology for the diagnosis of Barrett's‐associated HGD and/or carcinoma, and moderate sensitivity for BE. Diagn. Cytopathol. 2003;29:130–135. © 2003 Wiley‐Liss, Inc.
Volatile needle and wood extracts of oriental spruce Picea orientalis (L.) Link
Authors: Uçar Günes , Balaban Mualla , Usta Mustafa 
Published: September 2003

AbstractVolatile needle and wood extracts of oriental spruce obtained from trees grown naturally in eastern Black Sea regions and in an cultivated stand near Istanbul were analysed by GC–MS. Despite considerable differences in both tree‐to‐tree and natural‐to‐cultivated aspects, the needle and wood extracts exhibit characteristic compounds, which might be of chemotaxonomic interest. The monoterpenes δ‐3‐carene, limonene and bornyl acetate dominate in the needles, whereas the appreciable amounts of labdadienol isomers and high content of diterpenoids characterize the wood extracts. Copyright © 2003 John Wiley & Sons, Ltd.
Effect of starch and amylopectin concentrations on volatile ﬂavour release from aqueous model food systems
Authors: van Ruth Saskia M. , King Carmel 
Published: September 2003

AbstractThe inﬂuence of starch and amylopectin (0, 1, 2, 3, 4 and 5% w/v) in aqueous model food systems was evaluated for 20 volatile ﬂavour compounds. To understand the inﬂuence of the gelling agents on the thermodynamic and kinetic component of volatile ﬂavour release, the release of six alcohols, ﬁve ketones, four esters, three aldehydes, one sulphur compound and one terpene was determined under equilibrium conditions and mouth conditions. For equilibrium analysis, the air/product partition coefﬁcients of the compounds were determined by equilibrium headspace gas chromatography. Under mouth conditions, the ﬂavour released in a model mouth system was analysed by gas chromatography–mass spectrometry. Both gelling agents and their concentration affected the release of the volatile compounds signiﬁcantly, resulting in a distortion of the ﬂavour composition. Under equilibrium conditions, starch showed greater ﬂavour retention than amylopectin. Under mouth conditions the opposite was observed; amylopectin affected ﬂavour release more than starch. Both functional group and chain length of the ﬂavour compounds inﬂuenced the effect of the gelling agent. Since the effects of the gelling agents differed under mouth and equilibrium conditions, it is conceivable that the kinetic component of ﬂavour release is of greater importance for the effect of these gelling agents on volatile ﬂavour release than the thermodynamic component. Copyright © 2003 John Wiley & Sons, Ltd.
Essential oils of Lippia grandis Schau.
Authors: Maia José Guilherme S. , Taveira Francisca Socorro N. , Andrade Eloisa Helena A. , da Silva Milton Helio L. , Zoghbi Maria das Graças B. 
Published: September 2003

AbstractThe essential oils of leaves and ﬁne stems of ﬁve specimens of Lippia grandis collected at different places in the Amazon were analysed by GC–MS. These oils presented two distint chemical proﬁles. The percentage of the main identiﬁed compounds varied with the plant part, season time and collection site: thymol (19.3–69.7%), 1,8‐cineole (22.2–56.0%), carvacrol (0.2–21.2%), (E)‐nerolidol (0.8–21.1%), α‐pinene (0.1–17.4%), p‐cymene (4.0–14.0%), thymol methyl ether (1.0–14.0%) and β‐caryophyllene (0.7–12.1%). Based on the results, we believe to have at least two chemical varieties for Lippia grandis in the Amazon: the thymol and 1,8‐cineole types. Copyright © 2003 John Wiley & Sons, Ltd.
Chemical variability of Artemisia vulgaris L. essential oils originated from the Mediterranean area of France and Croatia
Authors: Jerkovic I. , Mastelic J. , Milos M. , Juteau F. , Masotti V. , Viano J. 
Published: September 2003

AbstractThe essential oils of Artemisia vulgaris L. originating from France and Croatia were subjected to detailed GC–MS analysis in order to determine possible similarities and differences between them and their chemical compositions, depending on the stage of development. Plant materials were collected in Provence (France) and Dalmatia (Croatia) at four different stages of development: vegetative (June), buds (July), ﬂowering (August) and seeding (September). Comparison of the chemical composition and content, depending on phenological stages in both localities, shows qualitative similarity, but also the noticeable difference in the amounts of most components. First, the chemical composition of main compounds is very different; Croatian oils are rich in hydrocarbons, which are absent or present in low amounts in French oils. Also, with regard to the yield of essential oils, the yields from Croatian oils were higher (0.09–0.61%) than these from French oil (0.04–0.15%) at each development stage, respectively. Copyright © 2003 John Wiley & Sons, Ltd.
Composition of the essential oil of Medicago marina L. from the coastal dunes of Tuscany, Italy
Authors: Flamini Guido , Luigi Cioni Pier , Morelli Ivano , Ceccarini Lucia , Andolfi Laura , Macchia Mario 
Published: September 2003

AbstractThis paper discusses essential oil composition of the dried aerial parts of Medicago marina harvested before and during blossom, in Vada (Livorno, Italy). In the two essential oils, rich in carbonylic compounds, 54 components were identified. Linalool (5.8%) and α‐terpineol (3.3%) were the main monoterpenes in the vegetative phase sample, while linalool (1.2%) and p‐mentha‐4‐en‐3‐one (0.4%) in the was the main constituent flowering stage. Sesquiterpenes were present only during the flowering stage, when the main constituents were non‐terpenic substances. 1‐Octen‐3‐ol and the (E)‐β‐ionone were the two constituents which showed the greater differences between the two samples (9.3% and 0.7% for the first component and 14.4% and 5.6% for the second component in the two different phases). Furthermore, phenyl propanoids were also identified in these oils, particularly methyl eugenol during the reproductive phase and eugenol during the vegetative phase. Copyright © 2003 John Wiley & Sons, Ltd.
Broad expression of fructose‐1,6‐bisphosphatase and phosphoenolpyruvate carboxykinase provide evidence for gluconeogenesis in human tissues other than liver and kidney
Authors: Yánez Alejandro J. , Nualart Francisco , Droppelmann Cristian , Bertinat Romina , Brito Mónica , Concha Ilona I. , Slebe Juan C. 
Published: September 2003

AbstractThe importance of renal and hepatic gluconeogenesis in glucose homeostasis is well established, but the cellular localization of the key gluconeogenic enzymes liver fructose‐1,6‐bisphosphatase (FBPase) and cytosolic phosphoenolpyruvate carboxykinase (PEPCK) in these organs and the potential contribution of other tissues in this process has not been investigated in detail. Therefore, we analyzed the human tissue localization and cellular distribution of FBPase and PEPCK immunohistochemically. The localization analysis demonstrated that FBPase was expressed in many tissues that had not been previously reported to contain FBPase activity (e.g., prostate, ovary, suprarenal cortex, stomach, and heart). In some multicellular tissues, this enzyme was detected in specialized areas such as epithelial cells of the small intestine and prostate or lung pneumocytes II. Interestingly, FBPase was also present in pancreas and cortex cells of the adrenal gland, organs that are involved in the control of carbohydrate and lipid metabolism. Although similar results were obtained for PEPCK localization, different expression of this enzyme was observed in pancreas, adrenal gland, and pneumocytes type I. These results show that co‐expression of FBPase and PEPCK occurs not only in kidney and liver, but also in a variety of organs such as the small intestine, stomach, adrenal gland, testis, and prostate which might also contribute to gluconeogenesis. Our results are consistent with published data on the expression of glucose‐6‐phosphatase in the human small intestine, providing evidence that this organ may play an important role in the human glucose homeostasis.
J. Cell. Physiol. 197: 189–197, 2003© 2003 Wiley‐Liss, Inc.
Intracellular dynamics of Smad‐mediated TGFβ signaling
Authors: Greene Robert M. , Nugent Paul , Mukhopadhyay Partha , Warner Dennis R. , Pisano M. Michele 
Published: September 2003

AbstractThe transforming growth factor‐β (TGFβ) family represents a class of signaling molecules that plays a central role in morphogenesis, growth, and cell differentiation during normal embryonic development. Members of this growth factor family are particularly vital to development of the mammalian secondary palate where they regulate palate mesenchymal cell proliferation and extracellular matrix synthesis. Such regulation is particularly critical since perturbation of either cellular process results in a cleft of the palate. While the cellular and phenotypic effects of TGFβ on embryonic craniofacial tissue have been extensively catalogued, the specific genes that function as downstream mediators of TGFβ action in the embryo during palatal ontogenesis are poorly defined. Embryonic palatal tissue in vivo and murine embryonic palate mesenchymal (MEPM) cells in vitro secrete and respond to TGFβ. In the current study, elements of the Smad component of the TGFβ intracellular signaling system were identified and characterized in cells of the embryonic palate and functional activation of the Smad pathway by TGFβ1, TGFβ2, and TGFβ3 was demonstrated. TGFβ‐initiated Smad signaling in cells of the embryonic palate was found to result in: (1) phosphorylation of Smad 2; (2) nuclear translocation of the Smads 2, 3, and 4 protein complex; (3) binding of Smads 3 and 4 to a consensus Smad binding element (SBE) oligonucleotide; (4) transactivation of transfected reporter constructs, containing TGFβ‐inducible Smad response elements; and (4) increased expression of gelatinases A and B (endogenous genes containing Smad response elements) whose expression is critical to matrix remodeling during palatal ontogenesis. Collectively, these data point to the presence of a functional Smad‐mediated TGFβ signaling system in cells of the developing murine palate. J. Cell. Physiol. 197: 261–271, 2003. © 2003 Wiley‐Liss, Inc.