UASt regulates expression of a Tag:V5-tagged Hsap\ATP5F1D cDNA clone (HsCD00506484, DNASU Plasmid Repository) bearing a p.Pro82Leu variant, which was identified as a homozygous mutation in a subject who presented with episodic lethargy, metabolic acidosis, 3-methylglutaconic aciduria, and hyperammonemia.