BCS1L and inborn mitochondrial metabolism disorder: Since the most severe cases of BCS1L-related mitochondrial diseases affect liver function, mostly leading to fatal metabolic syndromes, we knocked-down Bcs1 expression in the fat body, the fly functional analogue of liver and white adipose tissue, by crossing homozygous ppl-gal4 flies with homozygous UAS-shBcs1 flies.