FITC标记的ATP结合蛋白家族7抗体
产品名称： FITC标记的ATP结合蛋白家族7抗体
英文名称： Anti-ABCB7/FITC
产品编号： HZ-12331R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-ABCB7/FITC Conjugated antibody
FITC标记的ATP结合蛋白家族7抗体
| 英文名称 | Anti-ABCB7/FITC |
| 中文名称 | FITC标记的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. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 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 | Human, Mouse, Rat, Dog, Pig, Rabbit, Sheep, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 83kDa |
| 细胞定位 | 细胞膜 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from Human ABCB7 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 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 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. 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 |
过氧化物酶体膜包含几个ATP结合盒（ABC）转运体，ABCD1-4，已知存在于人过氧化物酶体膜（1）中。四种蛋白均为ABC半转运蛋白，二聚体形成活性转运蛋白（1）。ABCD1的突变导致X连锁肾上腺脑白质营养不良（X-ALD），这是一种影响脂质储存（2，3）的过氧化物酶体紊乱。ABCD2在小鼠中在脑和肾上腺器官中高表达，在X-ALD（4）中受到不利影响。过氧化物酶体膜包括2个定量的主要蛋白PMP22和ABCD3（5）。ABCD3与不规则形状的囊泡相关，该囊泡可能是有缺陷的过氧化物酶体或过氧化物酶体前体（5）。ABCD4定位为过氧化物酶体（1）。编码ABCD1—4的基因分别定位于人类染色体XQ28、12q11-q12、1p2-2-p21和14q24.3（3，6～8）。ABCB7是一个半转运体，参与血红素从线粒体到胞浆的转运，并映射到人类染色体Xq13.1-q13.3(9)。