ATP结合蛋白家族7抗体
规格：1mg/1ml
英文名： ABCB7
别名： ABC transporter 7 protein; ABC7; Abcb7; ABCB7_HUMAN; ASAT; Atm1p; ATP binding cassette 7; ATP binding cassette sub family B (MDR/TAP) member 7; ATP binding cassette sub family B member 7; ATP binding
分子量： 83kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from Human ABCB7
交叉反应：Human, Mouse, Rat, Dog, Pig, Rabbit, Sheep,
细胞定位：细胞浆 细胞膜
ATP结合蛋白家族7抗体产品介绍：background: The peroxisomal membrane contains several ATP-binding cassette (ABC) transporters, ABCD1–4 that are known to be present in the human peroxisome membrane (1). All four proteins are ABC half-transporters, which dimerize to form an active transporter (1). A mutation in the ABCD1 causes X-linked adrenoleukodystrophy (X-ALD), a peroxisomal disorder which affects lipid storage (2,3). ABCD2 in mouse, is expressed at high levels in the brain and adrenal organs, which are adversely affected in X-ALD (4). The peroxisomal membrane comprises 2 quantitatively major proteins, PMP22 and ABCD3 (5). ABCD3 is associated with irregularly shaped vesicles which may be defective peroxisomes or peroxisome precursors (5). ABCD4 localizes to peroxisomes (1). The genes which encode ABCD1–4 map to ATP结合蛋白家族7抗体human chromosome Xq28, 12q11-q12, 1p22-p21 and 14q24.3, respectively (3,6–8). ABCB7 is a half-transporter involved in the transport of heme from the mitochondria to the cytosol and maps to human chromosome Xq13.1-q13.3 (9). Function: Could be involved in the transport of heme from the mitochondria to the cytosol. Plays a central role in the maturation of cytosolic iron-sulfur (Fe/S) cluster-containing proteins. Subunit: Homodimer or heterodimer (Potential). Subcellular Location: Mitochondrion inner membrane. DISEASE: Defects in ATP结合蛋白家族7抗体ABCB7 are the cause of X-linked sideroblastic anemia with ataxia (ASAT) [MIM:301310]. ASAT is a recessive disorder characterized by an infantile to early childhood onset of nonprogressive cerebellar ataxia and mild anemia with hypochromia and microcytosis. Similarity: Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily. Contains 1 ABC transmembrane type-1 domain. Contains 1 ABC transporter domain. Gene ID: 22 Database links: Entrez Gene: 22 Human Entrez Gene: 11306 Mouse Entrez Gene: 302395 Rat Omim: 300135 Human SwissProt: O75027 Human SwissProt: Q61102 Mouse SwissProt: Q704E8 Rat Unigene: 370480 Human Unigene: 426128 Mouse Unigene: 20068 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
ATP结合蛋白家族7抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 信号转导 干细胞 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid