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PMID: 8256860 Published · ppublish English Journal Article Research Support, U.S. Gov't, P.H.S.

Overexpression and abnormal modification of the stress proteins alpha B-crystallin and HSP27 in Alexander disease.

The American journal of pathology ·Vol. 143 ·No. 6 ·1993-12-00 ·Pages 1743-53

Head MW, Corbin E, Goldman JE

Abstract

Alexander disease is a leukodystrophy characterized by the presence of numerous Rosenthal fibers, inclusion bodies in astrocytes. A major component of Rosenthal fibers is alpha B-crystallin, some of which is ubiquitinated. In this report, we show that Alexander central nervous system (CNS) tissues contain elevated messenger RNA and protein levels of both alpha B-crystallin and the related small heat shock protein, hsp27, and that Rosenthal fibers contain hsp27. The alpha B-crystallin and hsp27 polypeptide isoform patterns of Alexander disease CNS are also distinct from those of control samples, suggesting that postranslational modifications may be involved in Rosenthal fiber formation. We advance the hypothesis that Rosenthal fibers may be regarded as stress protein inclusions formed in astrocytes as part of a chronic stress response to an as yet unknown stimulus in the CNS of Alexander patients.

MeSH Terms
Astrocytes/chemistry,metabolism,pathology Blotting, Northern Blotting, Western Central Nervous System/chemistry,metabolism,pathology Central Nervous System Diseases/metabolism,pathology Child Crystallins/analysis,metabolism Heat-Shock Proteins/analysis,metabolism Humans Immunohistochemistry Infant Male Middle Aged RNA, Messenger/analysis,genetics
Chemicals
Crystallins Heat-Shock Proteins RNA, Messenger
Authors & Affiliations
3 authors, click to expand affiliations / ORCID
Head M W
Department of Pathology, Columbia University College of Physicians and Surgeons, New York, New York.
Corbin E
Goldman J E
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46 references, click to expand
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Article Info
Journal
The American journal of pathology
Abbr.
Am J Pathol
ISSN
0002-9440
Published
1993-12-00
Pages
1743-53
Language
English
Region
United States
NLM ID
0370502
PMCID
PMC1887278
Subset
IM
Grants
NEI NIH HHS · EY-09331 · United States
External Links
PubMed source PMC Full Text View journal details
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