The ATP5A1 gene encodes the α subunit of mitochondrial ATP synthase which produces ATP from ADP in the presence of a proton gradient across the membrane. The mitochondrial ATP synthase, also known as Complex V or F1F0 ATP synthase, is a multi-subunit enzyme complex consisting of two functional domains, the F1-containing the catalytic core and the Fo- containing the membrane proton channel. F0 domain has 10 subunits: a,b, c, d, e, f, g, OSCP, A6L, and F6. F1 is composed of subunits α, β, γ, δ, ε, and a loosely attached inhibitor protein IF1. Recently defect in ATP5A1 has been linked to the fatal neonatal mitochondrial encephalopathy. ATP5A1 is localized in the mitochondria and anti-ATP5A1 can be used as the internal control for mitochondrial or Complex V proteins. This antibody recognizes the endogenous ATP5A1 protein in lysates from various cell lines and tissues.