MGI:1918007Homozygotes for a knock-out allele die around E9.5 and show developmental arrest, cardiovascular defects, altered mitochondrial function and structure, and accumulation of oxidized cardiolipin. Homozygotes for a hypomorphic allele die within 1-4 weeks of life from sudden growth arrest followed by cachexia and lethargy, and show hypoglycemia, hyperlactatemia, and impaired mitochondrial efficiency in the kidney and liver.