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Mitochondrial hydroxyapatite deposits in spinocerebellar degeneration
Author(s) -
Munoz D. G.,
Emery E. S.,
Highland R. A.
Publication year - 1987
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410220211
Subject(s) - mitochondrion , degeneration (medical) , pathology , spinocerebellar ataxia , electron microscope , ataxia , calcium , chemistry , anatomy , microbiology and biotechnology , biology , medicine , neuroscience , physics , optics
We report the presence of crystalline deposits of calcium hydroxyapatite in the mitochondria of 2 children with sporadic spinocerebellar degeneration. The deposits, identified by electron microscopy, were found in the mitochondria of neurons and smooth muscle cells in one patient and in only smooth muscle cells in the second child, but not in other cell types. The calcific nature of the deposits was confirmed by laser microprobe mass analysis. The calcium overload may interfere with mitochondrial function, as has been shown in the cardiomyopathic strain of the Syrian hamster, a model of the cardiomyopathy of Friedreich's ataxia.