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PMID: 8382896 Published · ppublish English Journal Article Research Support, U.S. Gov't, P.H.S.

Cerebellar degeneration in the Niemann-Pick type C mouse.

Acta neuropathologica ·Vol. 85 ·No. 2 ·1993-00-00 ·Pages 175-84

Higashi Y, Murayama S, Pentchev PG, Suzuki K

Abstract

Chronological morphological changes and topographical distribution of degenerating Purkinje cells were studied in the murine model of Niemann-Pick disease type C (NPC mouse). Loss of Purkinje cells can be detected in the anterior vermis as early as 60 days of age, coinciding with early neurological signs, and progressed to total absence in the entire hemisphere and vermis with exception of nodules. Ultrastructurally, concentric lamellar inclusions were detected in the perikarya of degenerating Purkinje cells as well as in the focally enlarged branching points of their dendrites. Calbindin immunocytochemistry demonstrated dendritic pathology characterized by irregular contour of dendritic trees and decreased number of dendritic spines. Ubiquitin immunoreactivity revealed granular reaction products in the perikarya, dendrites and axons of Purkinje cells. Our studies demonstrated unique pathological features of Purkinje cells that involve perikarya, dendrites and axons in the NPC mouse.

MeSH Terms
Animals Calbindins Cerebellum/chemistry,ultrastructure Immunohistochemistry Mice Mice, Neurologic Mutants Microscopy, Electron Niemann-Pick Diseases/complications,metabolism S100 Calcium Binding Protein G/analysis Spinocerebellar Degenerations/etiology,metabolism Ubiquitins/analysis
Chemicals
Calbindins S100 Calcium Binding Protein G Ubiquitins
Authors & Affiliations
4 authors, click to expand affiliations / ORCID
Higashi Y
Department of Pathology (Neuropathology), University of North Carolina, Chapel Hill 27599-7525.
Murayama S
Pentchev P G
Suzuki K
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Article Info
Journal
Acta neuropathologica
Abbr.
Acta Neuropathol
ISSN
0001-6322
DOI
10.1007/BF00227765
Published
1993-00-00
Pages
175-84
Language
English
Region
Germany
NLM ID
0412041
Subset
IM
Grants
NIEHS NIH HHS · ES-01104 · United States
NICHD NIH HHS · HD-03110 · United States
NINDS NIH HHS · NS-24453 · United States
External Links
PubMed source DOI: 10.1007/BF00227765 View journal details
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