Predicted to enable acyl binding activity; acyl carrier activity; and mitochondrial large ribosomal subunit binding activity. Predicted to be involved in [2Fe-2S] cluster assembly; mitochondrial respiratory chain complex I assembly; and protein lipoylation. Located in mitochondrion. Part of respiratory chain complex I. Is active in mitochondrial inner membrane. Is expressed in several structures, including alimentary system; genitourinary system; integumental system; nervous system; and sensory organ. Orthologous to human NDUFAB1 (NADH:ubiquinone oxidoreductase subunit AB1).
MGI Description
PHENOTYPE: Skeletal muscle-specific knock-out leads to smaller body size, hypoglycemia, hypoinsulinemia, reduced muscle and liver glycogen levels, disrupted mitochondrial cristae and premature death within five days after birth. Overexpression increases glucose metabolism and protects against diet-induced obesity. [provided by MGI curators]