ISSN:
1573-4919
Keywords:
creatine kinase
;
mitochondrial myopathies
;
ragged red fibers
;
intramitochondrial inclusions
;
creatine analogues
;
phosphagen
Source:
Springer Online Journal Archives 1860-2000
Topics:
Biology
,
Chemistry and Pharmacology
,
Medicine
Notes:
Abstract Currently, considerable research activities are focussing on biochemical, physiological and pathological aspects of the creatine kinase (CK) — phosphorylcreatine (PCr) — creatine (Cr) system (for reviews see [1, 2]), but only little effort is directed towards a thorough investigation of Cr metabolism as a whole. However, a detailed knowledge of Cr metabolism is essential for a deeper understanding of bioenergetics in general and, for example, of the effects of muscular dystrophies, atrophies, CK deficiencies (e.g. in transgenic animals) or Cr analogues on the energy metabolism of the tissues involved. Therefore, the present article provides a short overview on the reactions and enzymes involved in Cr biosynthesis and degradation, on the organization and regulation of Cr metabolism within the body, as well as on the metabolic consequences of 3-guanidinopropionate (GPA) feeding which is known to induce a Cr deficiency in muscle. In addition, the phenotype of muscles depleted of Cr and PCr by GPA feeding is put into context with recent investigations on the muscle phenotype of ‘gene knockout’ mice deficient in the cytosolic muscle-type M-CK.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF01267947
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