Human adipose-derived mesenchymal stem cells as a new model of spinal and bulbar muscular atrophy

Spinal and bulbar muscular atrophy (SBMA) or Kennedy's disease is an X-linked CAG/polyglutamine expansion motoneuron disease, in which an elongated polyglutamine tract (polyQ) in the N-terminal androgen receptor (ARpolyQ) confers toxicity to this protein. Typical markers of SBMA disease are ARp...

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Veröffentlicht in:	PloS one 2014-11, Vol.9 (11), p.e112746
Hauptverfasser:	Dossena, Marta, Bedini, Gloria, Rusmini, Paola, Giorgetti, Elisa, Canazza, Alessandra, Tosetti, Valentina, Salsano, Ettore, Sagnelli, Anna, Mariotti, Caterina, Gellera, Cinzia, Navone, Stefania Elena, Marfia, Giovanni, Alessandri, Giulio, Corsi, Fabio, Parati, Eugenio Agostino, Pareyson, Davide, Poletti, Angelo
Format:	Artikel
Sprache:	eng
Schlagworte:	Adipocytes Adipocytes - metabolism Adipocytes - pathology Adipose Tissue - metabolism Adipose Tissue - pathology Aged Androgen receptors Androgens Animal models Antigens Atrophy Biocompatibility Biology and Life Sciences Biomedical materials Bone marrow Case-Control Studies Cell culture Cell Differentiation Chaperones Chondrocytes Disease Elongation Gene Expression Regulation Genes HSP70 Heat-Shock Proteins - genetics HSP70 Heat-Shock Proteins - metabolism Hsp70 protein Humans Inclusion bodies Inclusions Kennedy's disease Laboratories Male Medicine and Health Sciences Mesenchymal stem cells Mesenchymal Stem Cells - metabolism Mesenchymal Stem Cells - pathology Mesenchyme Metabolic disorders Middle Aged Models, Biological Molecular modelling Muscular Disorders, Atrophic - genetics Muscular Disorders, Atrophic - metabolism Muscular Disorders, Atrophic - pathology Neurobiology Neurosciences Neurotoxicity Nuclear transport Osteocytes Patients Peptides - genetics Peptides - metabolism Polyglutamine diseases Primary Cell Culture Proteasome Endopeptidase Complex - metabolism Proteasomes Proteins Receptors, Androgen - genetics Receptors, Androgen - metabolism Rodents Smooth muscle Spinal and bulbar muscular atrophy Stem cell transplantation Stem cells Toxicity Toxicity testing Translocation Trinucleotide repeat diseases Trinucleotide repeats Ubiquitin Ubiquitin - genetics Ubiquitin - metabolism
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