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Mitochondrial creatine kinase: a major constituent of pathological inclusions seen in mitochondrial myopathies.

Abstract：

:Overaccumulation of abnormally organized mitochondria in so-called "ragged-red" skeletal muscle fibers is a morphological hallmark of mitochondrial myopathies, in particular of mitochondrial encephalomyopathies. Characteristic for the abnormal mitochondria is the occurrence of highly ordered crystalline inclusions. Immuno-electron microscopy revealed that these inclusions react heavily with specific antibodies against mitochondrial creatine kinase (Mi-CK). Image processing of selected crystalline inclusions, sectioned along the crystallographic b, c planes, resulted in an averaged picture displaying an arrangement of regular, square-shaped particles with a central cavity. The overall appearance, dimensions, and symmetry of these building blocks are very reminiscent of single isolated Mi-CK octamers. Taking these findings together, it is concluded that Mi-CK octamers indeed represent the major, if not the only, component of these mitochondrial inclusions.

journal_name

Proc Natl Acad Sci U S A

journal_title

Proceedings of the National Academy of Sciences of the United States of America

authors

Stadhouders AM,Jap PH,Winkler HP,Eppenberger HM,Wallimann T

doi

10.1073/pnas.91.11.5089

subject

Has Abstract

pub_date

1994-05-24 00:00:00

pages

5089-93

issue

11

eissn

0027-8424

issn

1091-6490

journal_volume

91

pub_type

杂志文章

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