Mitochondrial creatine kinase: a major constituent of pathological inclusions seen in mitochondrial myopathies
| Autor: | Ad M. Stadhouders, Theo Wallimann, Hans M. Eppenberger, Paul H.K. Jap, Hanspeter Winkler |
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| Jazyk: | angličtina |
| Rok vydání: | 1994 |
| Předmět: |
Inclusion Bodies
Multidisciplinary Abnormal mitochondria Mitochondrial Myopathies Skeletal Muscle Fibers Mitochondrion Biology medicine.disease Cell biology Mitochondria Specific antibody Mitochondrial myopathy Biochemistry Mitochondrial Creatine Kinase medicine Humans Microscopy Immunoelectron Pathological Creatine Kinase Mitochondrial Encephalomyopathies Research Article |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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