Molecular and Cellular Biochemistry

, Volume 133, Issue 1, pp 235–243

Expression of the mitochondrial creatine kinase genes

  • R. Mark Payne
  • Arnold W. Strauss
Molecular Biology of Creatine Kinases

DOI: 10.1007/BF01267957

Cite this article as:
Payne, R.M. & Strauss, A.W. Mol Cell Biochem (1994) 133: 235. doi:10.1007/BF01267957


Mitochondrial Creatine Kinase (MtCK) is responsible for the transfer of high energy phosphate from mitochondria to the cytosolic carrier, creatine, and exists in mammals as two isoenzymes encoded by separate genes. In rats and humans, sarcomere-specific MtCK (sMtCK) is expressed only in skeletal and heart muscle, and has 87% nucleotide identity across the 1257 bp coding region. The ubiquitous isoenzyme of MtCK (uMtCK) is expressed in many tissues with highest levels in brain, gut, and kidney, and has 92% nucleotide identity between the 1254 bp coding regions of rat and human. Both genes are highly regulated developmentally in a tissue-specific manner. There is virtually no expression of sMtCK mRNA prior to birth. Unlike cytosolic muscle CK (MCK) and brain CK (BCK), there is no developmental isoenzyme switch between the MtCKs. Cell culture models representing the tissue-specific expression of either sMtCK or uMtCK are available, but there are no adequate developmental models to examine their regulation. Several animal models are available to examine the coordinate regulation of the CK gene family and include 1) Cardiac Stress by coarctation (sMtCK, BCK, and MCK), 2) Uterus and placenta during pregnancy (uMtCK and BCK), and 3) Diabetes and mitochondrial myopathy (sMtCK, BCK, and MCK). We report the details of these findings, and discuss the coordinate regulation of the genes necessary for high-energy transduction.

Key words

creatine kinasemitochondriametabolismcreatine phosphate shuttlegene expressionmuscle

Copyright information

© Kluwer Academic Publishers 1994

Authors and Affiliations

  • R. Mark Payne
    • 1
  • Arnold W. Strauss
    • 1
    • 2
  1. 1.Department of PediatricsWashington University School of MedicineSt. LouisUSA
  2. 2.Department of Molecular Biology and PharmacologyWashington University School of MedicineSt. LouisUSA