Nucleotide Binding Protein Like Protein (NUBPL)

NUBPL contains an N-terminal mitochondrial targeting sequence, a highly conserved nucleotide-binding domain, and a putative Fe/S-binding signature (CxxC). Western blot analysis of various human tissues revealed highest IND1 expression in liver and kidney, with lower expression in small intestine and brain. Immunoelectron microscopy revealed that knockdown of IND1 in HeLa cells via short hairpin RNA resulted in ultrastructural changes in mitochondria, including loss of crista membranes, massive remodeling of the respiratory supercomplexes, and increased lactate production, likely due to defective respiratory chain activity. IND1 depletion resulted in massive losses in several subunits of the peripheral arm of complex I, with the concomitant appearance of a 450-kD subcomplex representing part of the membrane arm.