Characterization of initiator and effector caspase expressions in dystrophinopathies.

Characterization of initiator and effector caspase expressions in dystrophinopathies. Research Abstract Details 

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Characterization of initiator and effector caspase expressions in dystrophinopathies. Abstract Text:

There is evidence that apoptotic cell death mechanisms contribute to muscle fiber loss in dystrophin-deficient muscle but there is little knowledge about the final degrading events of muscle fiber apoptosis. In muscle biopsy specimens from 14 patients with a dystrophinopathy (10 patients with DMD, two with Becker MD, two DMD carriers), expression of APAF-1 and caspase-9, upstream members of the apoptotic protease cascade, as well as of the downstream executioners caspase-2, -6 and -7, were studied by immunohistochemistry and Western blots. Besides predominant immunoreactivity in regenerating muscle fibers, which may contribute to apoptotic events during new muscle fiber formation, caspase-9, -6 and -7 displayed upregulation in non-regenerating, light microscopically intact but atrophic muscle fibers. Western blot analyses confirmed the upregulations. These findings indicate that, once activated, caspase-9 initiates a proteolytic, muscle fiber degrading cascade involving the downstream executioners caspase-6 and -7. However, lacking coexpression of APAF-1 suggests the existence of other pathways of caspase-9 activation than through the "apoptosome" in dystrophinopathies.

Characterization of initiator and effector caspase expressions in dystrophinopathies. Publishing Authors By Initials

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Characterization of initiator and effector caspase expressions in dystrophinopathies. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Neuropathology : official journal of the Japanese

VOLUME: 26

Page Numbers: 24-31

Journal Abbreviation: Neuropathology

ISSN: 0919-6544

DAY: 10

MONTH: Feb

YEAR: 2006

Characterization of initiator and effector caspase expressions in dystrophinopathies. Information

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LANGUAGE: eng

NlmUniqueID: 9606526

Characterization of initiator and effector caspase expressions in dystrophinopathies. Keywords Mesh Terms:

KEYWORDS: Proteins

MESH TERMS: metabolism

Chemical & Substance for Abstract: Characterization of initiator and effector caspase expressions in dystrophinopathies. Information

Substance Name: Caspases

Registry Number: EC 3.4.22.-

Grant and Affiliation Information for Characterization of initiator and effector caspase expressions in dystrophinopathies.

AFFILIATION: Neurological (Edinger-) Institute, Johann Wolfgang Goethe University Hospital, Frankfurt/M, Germany. dominique-suzanne.tews@kgu.de

Country: Australia

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MEDLINETA: Neuropathology

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