Fig. 2.

HIGD2A is up-regulated in lymphoblasts with a specific defect in the translation of mtDNA-encoded complex IV subunits. Volcano plot comparing mitochondrial protein abundances for proteins quantified in AARS2 lymphoblasts relative to control lymphoblasts. Subunits of complexes I, III and IV are colored as indicated. The curved line indicates significantly altered protein abundance determined through a false discovery rate-based approach (5% FDR, s0 = 0.1). n = 2 subcultures for the patient cells and n = 3 independent controls.