靶点背景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 loading control for mitochondrial or Complex V proteins. This antibody recognizes the endogenous ATP5A1 protein in lysates from various cell lines and tissues. The predicted MW of ATP5A1 is 60 kDa, while it undergoes the transit peptide cleavage to become a mature form around 50-55 kDa. Several isoforms of ATP5A1 exist due to the alternative splicing.
