Mar 11, 2023 · MDR3由ABCB4编码，主要在肝细胞的小管膜上表达并充当磷脂转运蛋白。 它的功能主要是将肝细胞内的磷脂酰胆碱转运到胆管腔，与胆汁酸混合后降低对胆管细胞毒性作用。 除此之外，在胆汁酸分泌过程中，由ATP8B1编码的FIC1将磷脂酰丝氨酸从肝细胞外翻转到胞内，这对抵抗高浓度的胆盐具有重要意义。 可见，ABCB4/ATP8B1突变可导致胆汁酸对胆管细胞损害加重。 目前已有研究 [4]表明，DILI、PFIC3、慢性肝病、ICP和低磷脂相关胆汁淤积症 (Lower …

Mar 5, 2024 · MDR3 deficiency is a rare genetic disorder that predominantly affects the liver. The disorder includes a spectrum of diseases that can range from mild to severe. The main symptom is interruption or suppression of the flow of bile from the liver (cholestasis). In addition, affected individuals may be prone to forming gallstones.

Multidrug resistance protein 3 (MDR3) is a hepatocyte canalicular membrane protein encoded by the ABCB4/MDR3 gene located on chromosome 7. Several liver diseases are known to be associated with MDR3 deficiency. The basic defect is reduced secretion of biliary phospholipid causing disturbance in the …

The expression of MDR3 mutants (or mutants for JAG1, FIC1, BSEP and TJP2) in cells that allow for easier quantification of function, perhaps in a high-throughput assay, will enable the rapid screen of pharmacologic agents to identify new drugs that …

Dec 24, 2024 · ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance as well as antigen presentation.

Oct 17, 2023 · 由ABCB4基因编码的多药耐药蛋白3（MDR3）是一种磷脂转运蛋白，主要表达在肝细胞的小管膜上，参与磷脂酰胆碱从肝细胞膜内向膜外的转位和小泡释放。 ABCB4突变导致的MDR3蛋白缺失或严重功能障碍可引起进行性家族性肝内胆汁淤积症3型（PFIC3）。 在PFIC3中，由于胆汁中磷脂酰胆碱缺乏，胆盐/胆汁酸无法与磷脂形成混合微粒，游离的胆盐通过其毒性去垢效应引起毛细胆管膜损伤，出现高γ-谷氨酰转移酶（GGT）胆汁淤积症。 胆汁酸/胆盐代谢紊 …

The ABCB4 gene (also known as MDR3) provides instructions for making a protein that helps move certain fats called phospholipids across the membranes of liver cells. Learn about this gene and related health conditions.

The MDR3 transporter, a ~140 kDa protein, has been predicted to be a floppase that translocates phosphatidylcholine (PC) from the inner to the outer leaflet of the canalicular membrane bilayer. Translocation of PC makes the biliary phospholipids available for extraction into the canalicular lumen by bile salt mixed micelles and therefore ...

Oct 1, 2015 · Multidrug resistance protein 3 (MDR3) is a hepatocyte canalicular membrane protein encoded by the ABCB4/MDR3 gene located on chromosome 7. Several liver diseases are known to be associated with MDR3 deficiency.

The multidrug resistance protein 3 (MDR3) is a glycoprotein encoded by adenosine triphosphate-binding cassette, subfamily B, member 4 (ABCB4) gene, and plays a role as a transporter required for biliary excretion of phospholipids.