ATP5A1 Antibody

ATP synthase alpha-subunit (ATP5A1) is a component of the mitochondrial complex V, which functions to generate ATP from ADP. Generation of ATP is catalyzed by ATP synthase. ATP synthase is composed of a soluble catalytic core, F1, and a membrane-spanning proton channel, F0. ATP5A1 is one of five different subunits that comprise the catalytic F1 core. Interestingly, ATP5A1 is genetically linked to neurodevelopmental diseases (1). Moreover, the genetically linked mutant forms of C9ORF72, the most common genetic causes of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), bind to ATP5A1 and alter its protein expression, suggesting that mitochondrial dysfunction may be a major driver of disease in C9ORF72-linked ALS/FTD (2). 1.Jonckheere, A.I. et al. (2013) Brain 136, 1544-54. 2.Choi, S.Y. et al. (2019) Nat Neurosci 22, 851-862.