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. 1994 May 24;91(11):5089–5093. doi: 10.1073/pnas.91.11.5089

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

A M Stadhouders 1, P H Jap 1, H P Winkler 1, H M Eppenberger 1, T Wallimann 1
PMCID: PMC43937  PMID: 8197190

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.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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