| Abnormalities of developing white matter in lysosomal storage diseases. | |
| | |
MedLine Citation:
|
PMID: 10499433 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Inborn metabolic errors causing lysosomal storage have well-recognized effects on neuronal function and morphology. In some classically "neuronal" storage diseases, however, neuroradiologic observations of infants have suggested a delay in central nervous system myelination based on persistently "immature" signal intensities monitored over time. This review summarizes reported neuropathologic evaluations of central white matter in infantile and juvenile patients and in corresponding animal models with lysosomal storage disorders. The observed neuropathology is examined in light of published studies of the biochemistry and microscopic anatomy of normal myelinogenesis. Finally, arguments are advanced that at least part of the deficiency of white matter is attributable to direct effects of the metabolic state on oligodendrocyte maturation and function, in addition to secondary effects on neurons and their axons. |
| | |
Authors:
|
R D Folkerth |
Related Documents
:
|
22255803 - Identifying neuron communities during a reach and grasp task using an unsupervised clus... 21880923 - Loss of mecp2 in substantia nigra dopamine neurons compromises the nigrostriatal pathway. 2172663 - Combination of anterograde tracing with phaseolus vulgaris-leucoagglutinin, retrograde ... |
Publication Detail:
|
Type: Journal Article; Review |
Journal Detail:
|
Title: Journal of neuropathology and experimental neurology Volume: 58 ISSN: 0022-3069 ISO Abbreviation: J. Neuropathol. Exp. Neurol. Publication Date: 1999 Sep |
Date Detail:
|
Created Date: 1999-10-07 Completed Date: 1999-10-07 Revised Date: 2005-11-16 |
Medline Journal Info:
|
Nlm Unique ID: 2985192R Medline TA: J Neuropathol Exp Neurol Country: UNITED STATES |
Other Details:
|
Languages: eng Pagination: 887-902 Citation Subset: IM |
Affiliation:
|
Department of Pathology (Neuropathology), Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Animals Brain / growth & development* Cell Aging / physiology Disease Models, Animal Humans Lysosomal Storage Diseases / physiopathology* Myelin Sheath / physiology Oligodendroglia / physiology |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Effects of peptides: a cross-listing of peptides and their central actions published in the journal ...
Next Document: The effect of aging on pars compacta of the substantia nigra in rhesus monkey.