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Titolo:
Accumulation of abnormal amounts of glycosaminoglycans in murine mucopolysaccharidosis type VII neural progenitor cells does not alter the growth rate or efficiency of differentiation into neurons
Autore:
Heuer, GG; Skorupa, AF; Alur, RKP; Jiang, KL; Wolfe, JH;
Indirizzi:
Univ Penn, Sch Vet Med, Dept Pathobiol, Philadelphia, PA 19104 USA Univ Penn Philadelphia PA USA 19104 Pathobiol, Philadelphia, PA 19104 USA Univ Penn, Sch Vet Med, Ctr Comparat Med Genet, Philadelphia, PA 19104 USAUniv Penn Philadelphia PA USA 19104 Med Genet, Philadelphia, PA 19104 USA Childrens Hosp Philadelphia, Joseph Stokes Jr Res Inst, Div Neurol, Philadelphia, PA 19104 USA Childrens Hosp Philadelphia Philadelphia PA USA 19104lphia, PA 19104 USA
Titolo Testata:
MOLECULAR AND CELLULAR NEUROSCIENCE
fascicolo: 1, volume: 17, anno: 2001,
pagine: 167 - 178
SICI:
1044-7431(200101)17:1<167:AOAAOG>2.0.ZU;2-X
Fonte:
ISI
Lingua:
ENG
Soggetto:
BETA-GLUCURONIDASE DEFICIENCY; CENTRAL-NERVOUS-SYSTEM; MEDIATED GENE-TRANSFER; STEM-CELLS; MPS-VII; HIPPOCAMPAL-NEURONS; LYSOSOMAL STORAGE; PRECURSOR CELLS; IN-VITRO; RETROVIRUS VECTOR;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
59
Recensione:
Indirizzi per estratti:
Indirizzo: Wolfe, JH Univ Penn, Sch Vet Med, Dept Pathobiol, 34th & Civ Ctr Blvd, Philadelphia,PA 19104 USA Univ Penn 34th & Civ Ctr Blvd Philadelphia PA USA 19104 9104 USA
Citazione:
G.G. Heuer et al., "Accumulation of abnormal amounts of glycosaminoglycans in murine mucopolysaccharidosis type VII neural progenitor cells does not alter the growth rate or efficiency of differentiation into neurons", MOL CELL NE, 17(1), 2001, pp. 167-178

Abstract

Mucopolysaccharidosis type VII (MPS VII) results from deficiencies in the gene encoding the lysosomal enzyme beta -glucuronidase (GUSB). To study howthe genetic and biochemical defects of MPS disease affect neural cell populations, neural progenitor cells (NPCs) were isolated from MPS VII mice andnormal littermates. After growth in culture, approximately 90% of cells from both genotypes were nestin positive, a marker for NPCs, and lacked markers associated with lineage commitment. The mutant NPCs contained elevated levels of undegraded glycosaminoglycans (GAGs), the substrate for GUSB. Transduction with a retrovirus-vector expressing normal GUSB resulted in correction of the biochemical defects. Because of the demonstrated roles that GAGs and proteoglycans have in NPC biology and neural development, we tested whether the alterations in GAG metabolism affected MPS VII NPC properties regulated by GAG-containing molecules. MPS VII NPC cultures had growth rates in response to FGF-2 that were similar to normal cultures and the efficiency of differentiation into neurons was the same as with normal cells. Thus, even though isolated NPCs accumulate abnormally high levels of GAGs, these two key developmental properties were not altered when the cells were examined outside the milieu of the diseased brain.

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Documento generato il 21/01/20 alle ore 06:50:11