Isolated Complex Iii Deficiency, also known as isolated mitochondrial respiratory chain complex iii deficiency, is related to mitochondrial complex iii deficiency and leigh syndrome. An important gene associated with Isolated Complex Iii Deficiency is BCS1L (BCS1 Homolog, Ubiquinol-Cytochrome C Reductase Complex Chaperone), and among its related pathways/superpathways are Metabolism and Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins.. Affiliated tissues include liver, and related phenotype is Decreased shRNA abundance.