| 产品编号 | bs-12331R |
| 英文名称 | ABCB7 |
| 中文名称 | ATP结合蛋白家族7抗体 |
| 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 cassette sub family B member 7 mitochondrial; ATP binding cassette transporter 7; ATP-binding cassette sub-family B member 7; ATP-binding cassette transporter 7; EST140535; MDR7; mitochondrial; Multidrug resistance protein 7; P-glycoprotein 7; PGP7. |
| 研究领域 | 细胞生物 信号转导 干细胞 新陈代谢 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | (predicted: Human,Mouse,Rat,Dog,Pig,Rabbit,Sheep) |
| 产品应用 | IHC-P=1:100-500, IHC-F=1:100-500, ICC=1:100-500, IF=1:100-500, ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 83kDa |
| 细胞定位 | 细胞浆 细胞膜 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from Human ABCB7: 201-300/752 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 |
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 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 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. SWISS: O75027 Gene ID: 22 Database links: Entrez Gene: 22 Human Entrez Gene: 11306 Mouse Omim: 300135 Human SwissProt: O75027 Human SwissProt: Q61102 Mouse Unigene: 370480 Human Unigene: 426128 Mouse Unigene: 20068 Rat |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
