Mitochondrial creatine kinase in human health and disease

Mitochondrial creatine kinase (MtCK), together with cytosolic creatine kinase isoenzymes and the highly diffusible CK reaction product, phosphocreatine, provide a temporal and spatial energy buffer to maintain cellular energy homeostasis. Mitochondrial proteolipid complexes containing MtCK form micr...

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Bibliographic Details
Published in:Biochimica et biophysica acta 2006-02, Vol.1762 (2), p.164-180
Main Authors: Schlattner, Uwe, Tokarska-Schlattner, Malgorzata, Wallimann, Theo
Format: Article
Language:English
Subjects:
Animals
Apoptosis
Biochemistry, Molecular Biology
Cardiomyopathy
Creatine Kinase
Creatine Kinase - chemistry
Creatine Kinase - genetics
Creatine Kinase - metabolism
Crystalline intra-mitochondrial inclusion
Disease
Energy metabolism
Gene Expression Regulation, Enzymologic
Health
Humans
Life Sciences
Mitochondria
Mitochondria - enzymology
Mitochondria - genetics
Neurodegenerative disease
Oxidative damage
Up-Regulation
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Summary:Mitochondrial creatine kinase (MtCK), together with cytosolic creatine kinase isoenzymes and the highly diffusible CK reaction product, phosphocreatine, provide a temporal and spatial energy buffer to maintain cellular energy homeostasis. Mitochondrial proteolipid complexes containing MtCK form microcompartments that are involved in channeling energy in form of phosphocreatine rather than ATP into the cytosol. Under situations of compromised cellular energy state, which are often linked to ischemia, oxidative stress and calcium overload, two characteristics of mitochondrial creatine kinase are particularly relevant: its exquisite susceptibility to oxidative modifications and the compensatory up-regulation of its gene expression, in some cases leading to accumulation of crystalline MtCK inclusion bodies in mitochondria that are the clinical hallmarks for mitochondrial cytopathies. Both of these events may either impair or reinforce, respectively, the functions of mitochondrial MtCK complexes in cellular energy supply and protection of mitochondria form the so-called permeability transition leading to apoptosis or necrosis.
ISSN:0925-4439
0006-3002
0167-4889
1879-260X
1878-2434
DOI:10.1016/j.bbadis.2005.09.004