{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}: Shimada, T.; Hiramatsu, N.; Kasai, A.; Mukai, M.; Okamura, M.; Yao, J.; Huang, T.; Tamai, M.; Takahashi, S.; Nakamura, T.; Kitamura, M.
{Year}: 2008
{Title}: Suppression of adipocyte differentiation by Cordyceps militaris through activation of the aryl hydrocarbon receptor
{URL}: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=18664595&query_hl=1
{Tag}: 0
{Star}: 0
{Journal}: Am J Physiol Endocrinol Metab
{Volume}: 295
{Issue}: 4
{Pages}: E859-67
{DOI}: 10.1152/ajpendo.90373.2008
{Date Displayed}: 2008 Oct
{Date}: 2008-10-01
{Type of Work}: Journal Article; Research Support, Non-U.S. Gov't
{Accession Number}: 18664595
{Keywords}: 3T3 Cells; Adipocytes/*drug effects/metabolism; Adipogenesis/drug effects; Alkaline Phosphatase/metabolism; Animals; Azo Compounds; Biological Markers; Blotting, Northern; Blotting, Western; Cell Differentiation/drug effects; Cordyceps/*chemistry; Dioxins/pharmacology; Endoplasmic Reticulum/enzymology; Formazans; Indicators and Reagents; Lipid Metabolism/drug effects; Mice; Receptors, Aryl Hydrocarbon/*drug effects
{Abstract}: Mycelial extracts have a wide range of biological activities that modulate functions of mammalian cells. In this report, we sought to identify antiadipogenic mycelia with the use of 3T3-L1 cells and found that the extract of Cordyceps militaris exclusively suppressed differentiation of 3T3-L1 preadipocytes into mature adipocytes without affecting cell viability. This inhibitory effect was dose dependent, reversible, and associated with 1) a decrease in lipid accumulation, 2) blunted induction of adipocyte markers including adiponectin, peroxisome proliferator-activated receptor-gamma, and CCAAT/enhancer binding protein-alpha, and 3) sustained expression of a preadipocyte marker, monocyte chemoattractant protein-1. C. militaris also significantly decreased accumulation of lipid and hypertrophy in mature adipocytes and preserved their response to insulin (phosphorylation of Akt) during prolonged culture. Subsequent experiments revealed that C. militaris has the potential to activate the aryl hydrocarbon receptor (AhR). In 3T3-L1 cells, treatment with AhR agonists including benzo[a]pyrene and 3-methylcholanthrene reproduced the antiadipogenic effect of C. militaris. Furthermore, dominant-negative inhibition of AhR abrogated the suppressive effect of C. militaris on adipocyte differentiation. These results suggest that C. militaris has the potential to interfere with adipocyte differentiation through activation   of AhR.
{Author Address}: Department of Molecular Signaling, Interdisciplinary Graduate School of Medicine   and Engineering, University of Yamanashi, Chuo, Yamanashi, Japan.
{Language}: eng

{Reference Type}: Journal Article
{Author}: Wang, Xiao-Liang; Yang, Rui-Heng; Yao, Yi-Jian
{Year}: 2011
{Title}: DEVELOPMENT OF MICROSATELLITE MARKERS FOR OPHIOCORDYCEPS SINENSIS (OPHIOCORDYCIPITACEAE) USING AN ISSR-TAIL-PCR METHOD
{Tag}: 0
{Star}: 0
{Journal}: AMERICAN JOURNAL OF BOTANY
{Volume}: 98
{Issue}: 12
{Pages}: E391-E394
{ISBN/ISSN}: 0002-9122
{Keywords}: GENETIC DIVERSITY; CORDYCEPS-SINENSIS; fungi; ISSR-TAIL-PCR; microsatellite; Ophiocordyceps sinensis; Ophiocordycipitaceae; simple sequence repeat marker
{Abstract}: Premise of the study: Microsatellite primers were developed for Ophiocordyceps sinensis, an endangered medicinal fungus endemic to the Tibetan Plateau, to investigate its genetic diversity and population structure.
Methods and Results: An inter-simple sequence repeat (ISSR)-thermal asymmetric interlaced (TAIL)-PCR method was established to develop microsatellite markers. A total of 30 perfect and imperfect microsatellites were identified in 48 individuals of O. sinensis from five provinces within China representing different populations. Seventeen loci were polymorphic with two to four alleles per locus, while 13 were monomorphic.
Conclusions: The results indicate that the microsatellite markers developed here may be used in studies of population genetics and conservation biology of O. sinensis. Furthermore, the ISSR-TAIL-PCR method is a simple strategy for microsatellite marker development.
{Author Address}: Chinese Acad Sci, State Key Lab Mycol, Inst Microbiol, Beijing 100101, Peoples R China; Chinese Acad Sci, Grad Univ, Beijing 100049, Peoples R China; Chinese Acad Sci, State Key Lab Mycol, Inst Microbiol, Beijing 100101, Peoples R China; Chinese Acad Sci, Grad Univ, Beijing 100049, Peoples R China; Chinese Acad Sci, State Key Lab Mycol, Inst Microbiol, Beijing 100101, Peoples R China
{Database Provider}: Web of Science SCI
{Language}: English
{Country}: Peoples R China; Peoples R China