Anti-ATP-binding cassette sub-family A member 3 antibody [3C9]

Supplementary info

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

Activity summary

ATP-binding cassette sub-family A member 3 (ABCA3) also known as Surfactant Metabolism Dysfunction Pulmonary 3 is a vital membrane protein weighing approximately 170 kDa. It belongs to the ATP-binding cassette (ABC) protein family which also includes family binding proteins that transport various molecules across cellular membranes. ABCA3 is mainly expressed in alveolar type II cells of the lung where it plays a significant mechanical role in surfactant metabolism. The protein facilitates the transport of lipids into lamellar bodies which are critical for surfactant storage and secretion.

Biological function summary

ABCA3 is essential for normal pulmonary function as it contributes to surfactant phospholipid homeostasis. It operates as a monomer and is part of a larger complex with other lipid-processing proteins. This interaction ensures the correct assembly and secretion of pulmonary surfactant which reduces surface tension in the alveoli and prevents alveolar collapse. The dysfunction or absence of ABCA3 can disrupt these processes compromising lung function.

Pathways

The role of ABCA3 is significant in the surfactant metabolism pathway and lipid transportation processes. The surfactant metabolism pathway ensures the availability of pulmonary surfactant necessary for efficient respiratory function. In these pathways ABCA3 intersects with other proteins such as ABCA1 and proteins involved in the phospholipid synthesis and transport processes ensuring lipid proteins are correctly assembled for surfactant formation.

Associated diseases and disorders

ABCA3 mutations associate with respiratory diseases such as Neonatal Respiratory Distress Syndrome and interstitial lung disease. These diseases often result from impaired surfactant production and secretion caused by ABCA3 deficiency. Mutations in this protein can also lead to dysfunctional interactions with partner proteins like Surfactant Protein B (SP-B) and Surfactant Protein C (SP-C) further implicating its role in disease pathology. Understanding the connection between ABCA3 and these proteins reveals potential therapeutic targets for treating associated pulmonary conditions.