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ABCA3 Plays an important role in the formation of pulmonary surfactant, probably by transporting lipids such as cholesterol. Defects in ABCA3 are the cause of pulmonary surfactant metabolism dysfunction type 3 (SMDP3); also called pulmonary alveolar proteinosis due to ABCA3 deficiency. A rare lung disorder due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress. Belongs to the ABC transporter superfamily. ABCA family. Note: This description may include information from UniProtKB.
Protein type: Membrane protein, integral; Membrane protein, multi-pass; Transporter; Transporter, ABC family
Chromosomal Location of Human Ortholog: 16p13.3
Cellular Component: extracellular space; intracellular membrane-bound organelle; membrane; plasma membrane
Molecular Function: ATPase activity, coupled to transmembrane movement of substances; lipid transporter activity; transporter activity
Biological Process: cellular protein metabolic process; lipid transport; response to drug; transmembrane transport; transport
Disease: Surfactant Metabolism Dysfunction, Pulmonary, 3
Reference #:  Q99758 (UniProtKB)
Alt. Names/Synonyms: ABC transporter 3; ABC-C; ABC-C transporter; ABC3; ABCA3; ATP-binding cassette 3; ATP-binding cassette sub-family A member 3; ATP-binding cassette transporter 3; ATP-binding cassette, sub-family A (ABC1), member 3; EST111653; LBM180; MGC166979; MGC72201; SMDP3
Gene Symbols: ABCA3
Molecular weight: 191,362 Da
Basal Isoelectric point: 7.55  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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