ATP7B Rabbit pAb (一抗) | Bioss

bs-1718R

农牧业/家禽抗体

ATP7B Rabbit pAb

铜转运蛋白质β链抗体

PWD; WC1; WD; WND; ATP7B_HUMAN; ATP7B; Copper pump 2; Wilson disease-associated protein; 7.2.2.8; ATPase copper transporting beta; ATPase, Cu++ transporting, beta polypeptide (Wilson disease); ATPase, Cu++ transporting, beta polypeptide; Wilson disease; copper-transporting ATPase 2

Rabbit

KLH conjugated synthetic peptide derived from human ATP7B: 1351-1465/1465

IgG

Liquid

affinity purified by Protein A

Polyclonal

161 kDa

1mg/ml

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

铜转运蛋白质β链是铜转运蛋白质家族中的一种，可调节细胞内铜离子水平的铜转运P型三磷酸腺苷酶，ATP7B是生物体内广泛存在的一种极为重要的细胞膜上的酶，它的功能主要是维持细胞内外的离子及渗透压平衡、跨膜电化学和细胞的能量代谢.

ATP7b is an important protein for copper transport and elimination of excess copper from the body. ATP7b transports metals in and out of cells using ATP. There are 3 known isoforms of the ATP7b gene; A is found in the liver, kidney, and brain, the shorter form B is found in brain tissue, and the third isoform, known as WND/140 KDA is found in mitochondria. Mutations in the ATP7b gene can cause Wilson's disease, an inherited disorder causing copper poisoning in the brain and liver.

ATP7B

BTLA

Monomer. Interacts with COMMD1/MURR1.

Golgi apparatus, trans-Golgi network membrane; Multi-pass membrane protein. Isoform 2: Cytoplasm. WND/140 kDa: Mitochondrion.

Most abundant in liver and kidney and also found in brain. Isoform 2 is expressed in brain but not in liver. The cleaved form WND/140 kDa is found in liver cell lines and other tissues.

Isoform 1 may be proteolytically cleaved at the N-terminus to produce the WND/140 kDa form.

Defects in ATP7B are the cause of Wilson disease (WD) [MIM:277900]. WD is an autosomal recessive disorder of copper metabolism in which copper cannot be incorporated into ceruloplasmin in liver, and cannot be excreted from the liver into the bile. Copper accumulates in the liver and subsequently in the brain and kidney. The disease is characterized by neurologic manifestations and signs of cirrhosis.

Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily.
Contains 6 HMA domains.

Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.