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ABCA3

Synonym: ATP-binding cassette sub-family A member 3, ABC-C
German: ABC-A3

1 Definition

ABCA3 (ATP-binding cassette sub-family A member 3) is a protein that belongs to the superfamily of ABC transport proteins and is located in the membrane of lamellar bodies in type II pneumocytes.

2 Biochemistry

The transport protein ABC A3 (1704 amino acids, 190 kDa) is synthesized by type II pneumocytes and also by cells of the heart, kidneys, brain, and pancreas.

Proteins of the ABC family utilize energy released by ATP hydrolysis to transport a broad range of substrates across biological membranes.

ABCA3 transports phospholipids (predominantly dipalmitoylphosphatidylcholine = lecithin) from the cytoplasm of type II pneumocytes to the lamellar bodies where phospholipids are stored for surfactant (surface-active agent) metabolism, and can be released through exocytosis when required.

3 Pathology

Mutations in ABCA3 can cause potentially lethal neonatal diseases (e.g. acute respiratory distress syndrome). The histological signs are hyperplasia of type II pneumocytes and alveolar macrophages that have a foamy appearance in case of ABCA3 mutations.

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