| Abnormalities in neural progenitor cells in a dog model of lysosomal storage disease. | |
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MedLine Citation:
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PMID: 17882020 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Lysosomal storage disorders constitute a large group of genetic diseases, many of which are characterized by mental retardation and other neurologic symptoms. The mechanisms of neural dysfunction remain poorly understood. Because neural progenitor cells (NPCs) are fundamentally important to normal brain development and function, we investigated NPC properties in a canine model of mucopolysaccharidosis VII (MPS VII). MPS VII is a lysosomal storage disorder characterized by defects in the catabolism of glycosaminoglycans. NPCs were isolated from the olfactory bulb, cerebellum, and striatal subventricular zone of normal and MPS VII (beta-glucuronidase-deficient) postnatal dog brains. Canine NPCs (cNPCs) from normal and MPS VII brains had similar growth curves, but cerebellar-derived cNPCs grew significantly slower than those derived from other regions. In differentiation assays, MPS VII cNPCs from the striatal subventricular zone and cerebellum generated fewer mature neuronal and/or glial cells than normal, and MPS VII olfactory bulb-derived cNPCs retained significantly more phenotypically immature cells. These differences were only present at the earliest time point after isolation; at later passages, there were no differences attributable to genotype. The data suggest that MPS VII cNPCs respond differently to developmental cues in vivo, probably because of the diseased neural microenvironment rather than intrinsic cellular deficits. |
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Authors:
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Raquel M Walton; John H Wolfe |
Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural |
Journal Detail:
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Title: Journal of neuropathology and experimental neurology Volume: 66 ISSN: 0022-3069 ISO Abbreviation: J. Neuropathol. Exp. Neurol. Publication Date: 2007 Aug |
Date Detail:
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Created Date: 2007-09-20 Completed Date: 2007-10-26 Revised Date: 2007-12-03 |
Medline Journal Info:
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Nlm Unique ID: 2985192R Medline TA: J Neuropathol Exp Neurol Country: United States |
Other Details:
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Languages: eng Pagination: 760-9 Citation Subset: IM |
Affiliation:
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W F Goodman Center for Comparative Medical Genetics, and Department of Pathology, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Animals, Newborn Cell Differentiation / genetics Cells, Cultured Cerebellum / pathology Cerebral Ventricles / growth & development, pathology* Disease Models, Animal Dogs Lysosomal Storage Diseases / genetics, pathology* Mucopolysaccharidosis VII / genetics* Neurons / pathology* Stem Cells / pathology* |
| Grant Support | |
ID/Acronym/Agency:
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DK42707/DK/NIDDK NIH HHS; DK46637/DK/NIDDK NIH HHS; DK63973/DK/NIDDK NIH HHS; RR02512/RR/NCRR NIH HHS; RR07063/RR/NCRR NIH HHS |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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