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Creatine transporter and mitochondrial creatine kinase protein content in myopathies
Author(s) -
Tarnopolsky M.A.,
Parshad A.,
Walzel B.,
Schlattner U.,
Wallimann T.
Publication year - 2001
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.1055
Subject(s) - creatine , myopathy , phosphocreatine , creatine kinase , mitochondrial myopathy , medicine , endocrinology , muscular dystrophy , skeletal muscle , duchenne muscular dystrophy , biology , biochemistry , mitochondrial dna , gene , energy metabolism
Total creatine or phosphocreatine, or both, are reduced in the skeletal muscle of patients with inflammatory myopathy, mitochondrial myopathy, and muscular dystrophy/congenital myopathy. We used Western blotting techniques to measure skeletal muscle creatine transporter protein and sarcomeric mitochondrial creatine kinase (mtCK) protein content in patients with inflammatory myopathy ( N = 8), mitochondrial myopathy ( N = 5), muscular dystrophy ( N = 7), and congenital myopathy ( N = 3), as compared to a control group without a neuromuscular diagnosis ( N = 8). Creatine transporter protein content was lower for all groups compared to control subjects ( P < 0.05; P < 0.01 for congenital myopathy). Mitochondrial CK (mtCK) was lower for inflammatory myopathy ( P < 0.05), higher for mitochondrial myopathy ( P < 0.05), not different for muscular dystrophy, and markedly lower for the congenital myopathy group ( P < 0.01), compared to control subjects. Together, these data suggest that the reduction in total creatine or phosphocreatine in patients with certain myopathies is correlated with creatine transporter and not mtCK protein content. This further supports the belief that creatine monohydrate supplementation may benefit patients with low muscle creatine stores, although the reduction in creatine transporter protein may have implications for dosing. © 2001 John Wiley & Sons, Inc. Muscle Nerve 24: 682–688, 2001