Title : Mitochondrial ATP-synthase deficiency in a child with 3-methylglutaconic aciduria.

Pub. Date : 1992 Dec

PMID : 1287564






1 Functional Relationships(s)
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1 Investigation of muscle mitochondrial function at 1 y of age revealed a severe mitochondrial ATP-synthase deficiency (oligomycin-sensitive, dinitrophenol-stimulated Mg2+ ATPase activity: 27 nmol x min-1 x (mg protein)-1, control range 223-673 nmol x min-1 x (mg protein)-1. Dinitrophenols mucin 7, secreted Homo sapiens