{Reference Type}: Journal Article
{Author}: Navarro, V.; Scott, C.; Briggs, T. A.; Barete, S.; Frances, C.; Lebon, P.; Maisonobe, T.; Rice, G. I.; Wouters, C. H.; Crow, Y. J.
{Year}: 2008
{Title}: Two further cases of spondyloenchondrodysplasia (SPENCD) with immune dysregulation
{URL}: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=18924170&query_hl=1
{Tag}: 0
{Star}: 0
{Journal}: Am J Med Genet A
{Volume}: 146A
{Issue}: 21
{Pages}: 2810-5
{DOI}: 10.1002/ajmg.a.32518
{Date Displayed}: 2008 Nov 1
{Date}: 2008-11-01
{Type of Work}: Case Reports; Journal Article
{Original Publication}: Copyright 2008 Wiley-Liss, Inc.
{Accession Number}: 18924170
{Keywords}: Adult; Autoimmune Diseases/diagnosis/*genetics/immunology; Brain/pathology; Child, Preschool; Consanguinity; Diagnosis, Differential; Female; Humans; Male; Myositis/pathology; Osteochondrodysplasias/diagnosis/*genetics/*immunology
{Abstract}: Although the diagnosis of spondyloenchondrodysplasia (SPENCD) can only be made in the presence of characteristic metaphyseal and vertebral lesions, a recent report has highlighted the pleiotropic manifestations of this disorder which include significant neurological involvement and variable immune dysfunction. Here we present two patients, one of whom was born to consanguineous parents, further illustrating the remarkable clinical spectrum of this disease. Although both patients demonstrated intracranial calcification, they were discordant for the presence of mental retardation, spasticity and white matter abnormalities. And whilst one patient had features consistent with diagnoses of Sjogren syndrome, polymyositis, hypothyroidism and severe scleroderma, the other patient had clinical manifestations and an autoantibody profile of systemic lupus erythematosus. These cases further illustrate the association of SPENCD with immune dysregulation and highlight the differential diagnosis with Aicardi-Goutieres syndrome and other disorders associated with the presence of intracranial calcification. Undoubtedly, identification of the underlying molecular and pathological basis of SPENCD will provide important insights into immune and skeletal regulation.
{Author Address}: AP-HP, Epilepsy Unit, Pitie-Salpetriere Hospital, Paris, France. vincent.navarro@psl.aphp.fr
{Language}: eng

{Reference Type}: Journal Article
{Author}: Wu, M.; Melichian, D. S.; Chang, E.; Warner-Blankenship, M.; Ghosh, A. K.; Varga, J.
{Year}: 2009
{Title}: Rosiglitazone abrogates bleomycin-induced scleroderma and blocks profibrotic responses through peroxisome proliferator-activated receptor-gamma
{URL}: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=19147827&query_hl=1
{Tag}: 0
{Star}: 0
{Journal}: Am J Pathol
{Volume}: 174
{Issue}: 2
{Pages}: 519-33
{DOI}: 10.2353/ajpath.2009.080574
{Date Displayed}: 2009 Feb
{Date}: 2009-02-01
{Type of Work}: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
{Accession Number}: 19147827
{Keywords}: Adipocytes/cytology; Animals; Antibiotics, Antineoplastic/toxicity; Bleomycin/toxicity; Cell Differentiation/drug effects; Cell Movement/drug effects; Collagen/drug effects; Female; Fibrosis/chemically induced/drug therapy/metabolism; Flow Cytometry; Fluorescent Antibody Technique; Gene Expression/drug effects; Hypoglycemic Agents/*pharmacology; Immunohistochemistry; Inflammation/chemically induced/drug therapy/metabolism; Mice; Mice, Inbred BALB C; PPAR gamma/drug effects/*metabolism; Reverse Transcriptase Polymerase Chain Reaction; Scleroderma, Systemic/chemically induced/*drug therapy/*metabolism; Stem Cells/cytology; Thiazolidinediones/*pharmacology; Transforming Growth Factor beta1/drug effects/metabolism
{Abstract}: The nuclear hormone receptor, peroxisome proliferator-activated receptor (PPAR)-gamma, originally identified as a key mediator of adipogenesis, is expressed widely and implicated in diverse biological responses. Both natural and synthetic agonists of PPAR-gamma abrogated the stimulation of collagen synthesis   and myofibroblast differentiation induced by transforming growth factor (TGF)-beta in vitro. To characterize the role of PPAR-gamma in the fibrotic process in vivo, the synthetic agonist rosiglitazone was used in a mouse model of scleroderma. Rosiglitazone attenuated bleomycin-induced skin inflammation and dermal fibrosis as well as subcutaneous lipoatrophy and counteracted the up-regulation of collagen gene expression and myofibroblast accumulation in the lesioned skin. Rosiglitazone treatment reduced the induction of the early-immediate transcription factor Egr-1 in situ without also blocking the activation of Smad2/3. In both explanted fibroblasts and skin organ cultures, rosiglitazone prevented the stimulation of collagen gene transcription and cell migration elicited by TGF-beta. Rosiglitazone-driven adipogenic differentiation of both fibroblasts and preadipocytes was abrogated in the presence of TGF-beta;   this effect was accompanied by the concomitant down-regulation of cellular PPAR-gamma mRNA expression. Collectively, these results indicate that rosiglitazone treatment attenuates inflammation, dermal fibrosis, and subcutaneous lipoatrophy via PPAR-gamma in a mouse model of scleroderma and suggest that pharmacological PPAR-gamma ligands, widely used as insulin sensitizers in the treatment of type-2 diabetes mellitus, may be potential therapies for scleroderma.
{Author Address}: Section of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago IL 60611, USA.
{Language}: eng

{Reference Type}: Journal Article
{Author}: Li, Xing-Tai; Li, Hong-Cheng; Li, Chun-Bin; Dou, De-Qiang; Gao, Ming-Bo
{Year}: 2010
{Title}: Protective Effects on Mitochondria and Anti-Aging Activity of Polysaccharides from Cultivated Fruiting Bodies of Cordyceps militaris
{Tag}: 0
{Star}: 0
{Journal}: AMERICAN JOURNAL OF CHINESE MEDICINE
{Volume}: 38
{Issue}: 6
{Pages}: 1093-1106
{ISBN/ISSN}: 0192-415X
{Keywords}: FREE-RADICAL THEORY; OXIDATIVE STRESS; ANTIOXIDANT ACTIVITIES; LIPID-PEROXIDATION; PERMEABILITY TRANSITION; CELL-FUNCTION; CYTOCHROME-C; RAT-LIVER; LIFE-SPAN; IN-VITRO; Cordyceps militaris Polysaccharides; Mitochondria; Anti-Aging; Reactive Oxygen Species; Antioxidase
{Abstract}: Cordyceps militaris (L.) Link is an entomopathogenic fungus parasitic to Lepidoptera larvae, and is widely used as a folk tonic or invigorant for longevity in China. Although C. militaris has been used in traditional Chinese medicine for millennia, there is still a lack convincing evidence for its anti-aging activities. This study was performed to investigate the effects of polysaccharides from cultivated fruiting bodies of C. militaris (CMP) on mitochondrial injury, antioxidation and anti-aging activity. Fruiting bodies of C. militaris were cultivated artificially under optimized conditions. The spectrophotometric method was used to measure thiobarbituric acid reactive substances (TBARS), mitochondrial swelling, and activities of scavenging superoxide anions in vitro. D-galactose (100 mg/kg/day) was injected subcutaneously into back of the neck of mice for 7 weeks to induce an aging model. The effects of CMP on the activities of catalase (CAT), surperoxide dismutase (SOD), glutathione peroxidase (GPx) and anti-hydroxyl radicals were assayed in vivo using commercial monitoring kits. The results showed that CMP could inhibit mitochondrial injury and swelling induced by Fe(2+)-L-Cysteine in a concentration-dependent manner and it also had a significant superoxide anion scavenging effect. Moreover, the activities of CAT, SOD, GPx and anti-hydroxyl radicals in mice liver were increased significantly by CMP. These results indicate that CMP protects mitochondria by scavenging reactive oxygen species (ROS), inhibiting mitochondrial swelling, and increasing the activities of antioxidases. Therefore, CMP may have pharmaceutical values for mitochondrial protection and anti-aging. CMP was the major bioactive component in C. militaris.
{Author Address}: Dalian Nationalities Univ, Coll Life Sci, Dalian 116600, Peoples R China; GeneSci Pharmaceut Co Ltd, Res & Dev Dept, High Tech Dev Zone, Changchun 130012, Peoples R China; Dalian Nationalities Univ, Coll Life Sci, Dalian 116600, Peoples R China; Liaoning Univ Tradit Chinese Med, Coll Pharm, Dalian 116600, Peoples R China; Dalian Nationalities Univ, Coll Life Sci, Dalian 116600, Peoples R China
{Database Provider}: Web of Science SCI
{Language}: English
{Country}: Peoples R China; Peoples R China; Peoples R China

{Reference Type}: Journal Article
{Author}: Wang, Ying; Wang, Min; Ling, Yun; Fan, Weiqiang; Wang, Yufeng; Yin, Hongping
{Year}: 2009
{Title}: Structural Determination and Antioxidant Activity of a Polysaccharide from the Fruiting Bodies of Cultured Cordyceps sinensis
{Tag}: 0
{Star}: 0
{Journal}: AMERICAN JOURNAL OF CHINESE MEDICINE
{Volume}: 37
{Issue}: 5
{Pages}: 977-989
{ISBN/ISSN}: 0192-415X
{Keywords}: FUNGUS; DEGRADATION; OXIDATION; MYCELIUM; EXTRACT; Cordyceps sinensis; Polysaccharide; Mycelium; Structure; Antioxidant Activity
{Abstract}: A water-soluble polysaccharide named CPS1 had been isolated from C. sinensis mycelium by hot water extraction, ethanol precipitation, anion-exchange, and gel-permeation chromatography. UV spectra, FTIR spectra, partial acid hydrolysis, PMP procolumn derivation, periodate oxidation and Smith degradation Studies were conducted to elucidate its structure. The results indicated that CPS1 was a glucomannogalactan with the monosaccharide composition of glucose: mannose: galactose = 2.8: 2.9: 1. The total carbohydrate content of CPS1 was 99.0%. The weight-average molecular weight wits 8.1 x 10(3) Da. The results predicted (1 -> 2) and (1 -> 4)-linkage of mannose, (1 -> 3)-linkage of galactose, (1 ->) and (1 -> 3, 6)-linkage of glucose composed the backbone of CPS1. CPS1 was also evaluated for its antioxidant activity in vitro, including scavenging effects on the hydroxyl radicals, the reducing power, Fe(2+)-chelating activity, scavenging effect on superoxide radicals, as well as the inhibition of hydrogen peroxide induced haemolysis. CPS1 showed a high antioxidant effect, especially scavenging effect of hydroxyl radicals, the reducing power and Fe(2+)-chelating activity. The results provide scientific support for the antioxidant activity and indicated a connection between antioxidant activity and reparation of renal failure.
{Author Address}: <AuCollectiveName>Wang, Ying</AuCollectiveName>
</fullauthorname>
<author>Wang, M</author>
<fullauthorname>
<AuRole>Author</AuRole>
<AuLastName>Wang</AuLastName>
<AuFirstName>Min</AuFirstName>
<address number="1">China Pharmaceut Univ, Coll Life Sci &amp; Technol, Sch Life Sci &amp; Technol, Dept Microbiol &amp; Biochem, Nanjing 210009, Jiangsu, Peoples R China
{Database Provider}: Web of Science SCI
{Language}: English
{Country}: Peoples R China