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Mitochondrial respiratory chain defects are not accompanied by an increase in the activities of lactate dehydrogenase or manganese superoxide dismutase in paediatric skeletal muscle biopsies
Author(s) -
Hargreaves I. P.,
Heales S. J. R.,
Land J. M.
Publication year - 1999
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1023/a:1005643508075
Subject(s) - lactate dehydrogenase , skeletal muscle , superoxide dismutase , respiratory chain , mitochondrial respiratory chain , electron transport chain , biochemistry , dismutase , succinate dehydrogenase , medicine , mitochondrion , endocrinology , biology , enzyme , chemistry
Both the activity of lactate dehydrogenase (LDH) and the quantity of manganese superoxide dismutase (MnSOD) protein have been reported to be increased in fibroblasts from individuals with mitochondrial electron transport chain defects. To ascertain whether this is a general phenomenon, we have determined the specific activities of these enzymes in skeletal muscle biopsies from control individuals and patients with defined electron transport chain defects. On investigation, both LDH and MnSOD activities were not found to be elevated. These findings suggest a possible fundamental difference between skeletal muscle preparations and fibroblasts with regard to their metabolic response to an electron transport chain defect.