返回首页

ABCA3 Q99758

磷脂转运ATP酶ABCA3 (EC 7.6.2.1) (ABC-C转运蛋白) (ATP结合盒亚家族A成员3) (ATP结合盒转运蛋白3) (ATP结合盒3) (异生素转运ATP酶ABCA3) (EC 7.6.2.2) [切割为:150 Kda成熟形式]

蛋白质信息 (UniProt)

磷脂转运ATP酶ABCA3 (EC 7.6.2.1) (ABC-C转运蛋白) (ATP结合盒亚家族A成员3) (ATP结合盒转运蛋白3) (ATP结合盒3) (异生素转运ATP酶ABCA3) (EC 7.6.2.2) [切割为:150 Kda成熟形式]

Q99758

功能描述

催化磷脂(如磷脂酰胆碱和磷酸甘油)从细胞质依赖ATP转运至板层小体的腔侧,进而参与板层小体的生物发生及肺表面活性剂的稳态(PubMed:16959783, PubMed:17574245, PubMed:27177387, PubMed:28887056, PubMed:31473345)。优先转运含有短酰基链的磷脂酰胆碱(PubMed:27177387)。此外,作为巨噬细胞膜上米替福新外排转运体发挥作用,并通过胞内囊泡转运游离胆固醇(FC),即作为表面活性剂成分将FC从细胞中清除,从而保护细胞免受游离胆固醇毒性(PubMed:25817392, PubMed:26903515, PubMed:27177387)。 {ECO:0000269|PubMed:16959783, ECO:0000269|PubMed:17574245, ECO:0000269|PubMed:25817392, ECO:0000269|PubMed:26903515, ECO:0000269|PubMed:27177387, ECO:0000269|PubMed:28887056, ECO:0000269|PubMed:31473345}。

组织特异性

Expressed in brain, pancreas, skeletal muscle and heart (PubMed:8706931). Highly expressed in the lung in an AT2-cell-specific manner (PubMed:11718719, PubMed:8706931). Weakly expressed in placenta, kidney and liver (PubMed:8706931). Also expressed in medullary thyroid carcinoma cells (MTC) and in C-cell carcinoma (PubMed:8706931).

亚细胞定位

Endosome, multivesicular body membrane

关键词

3D-structure Alternative splicing ATP-binding Cytoplasmic vesicle Disease variant Disulfide bond Endosome Glycoprotein Lipid transport Lysosome