Anti-ATPAF1 antibody

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

ATPAF1 also known as ATP Synthase Mitochondrial F1 Complex Assembly Factor 1 functions as a mitochondrial chaperone assisting in the assembly of ATP synthase. This protein weighs approximately 35 kDa. Its expression occurs mainly in tissues with high energy demands such as the heart and skeletal muscle. ATPAF1 helps ensure proper M-ATPase complex formation essential for cellular ATP production.

Biological function summary

ATPAF1 supports mitochondrial energy metabolism by participating in assembling and stabilizing the ATP synthase complex also referred to as Complex V. Without ATPAF1 cells struggle to maintain efficient ATP synthesis due to improper complex assembly. It partners with other assembly factors such as ATPAF2 to facilitate the construction and proper function of this critical mitochondrial enzyme.

Pathways

ATPAF1 operates within the oxidative phosphorylation pathway serving an important role in ATP generation. This pathway drives most cellular processes by producing ATP through electron transport and chemiosmosis. ATPAF1 relates closely to the mitochondrial proteins involved in the electron transport chain particularly with ATP5F1 which forms part of ATP synthase's core structure.

Associated diseases and disorders

ATPAF1 has links to mitochondrial diseases such as Leigh syndrome and mitochondrial myopathy. Patients with defects in ATPAF1 or related proteins may experience issues in energy-dependent tissues leading to symptoms like muscle weakness and neurological dysfunction. ATPAF1 interacts with other proteins like DNAJC19 which also associates with mitochondrial integrity and energy metabolism disorders.