ABCB7 Polyclonal Antibody
- 货号:YT0046
- 应用:WB;IHC;IF;ELISA
- 种属:Human;Rat;Mouse;
- 蛋白名称:
- ATP-binding cassette sub-family B member 7 mitochondrial
- 免疫原:
- The antiserum was produced against synthesized peptide derived from human ABCB7. AA range:691-740
- 特异性:
- ABCB7 Polyclonal Antibody detects endogenous levels of ABCB7 protein.
- 组成:
- Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- 来源:
- Polyclonal, Rabbit,IgG
- 稀释:
- WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200
- 纯化工艺:
- The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
- 储存:
- -15°C to -25°C/1 year(Do not lower than -25°C)
- 其他名称:
- ABCB7;ABC7;ATP-binding cassette sub-family B member 7; mitochondrial;ATP-binding cassette transporter 7;ABC transporter 7 protein
- 实测条带:
- Human:83kD,Mouse/Rat 100kD
- 背景:
- The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. 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. This gene encodes a half-transporter involved in the transport of heme from the mitochondria to the cytosol. With iron/sulfur cluster precursors as its substrates, this protein may play a role in metal homeostasis. Mutations in this gene have been associated with mitochondrial iron accumulation and isodicentric (X)(q13) and sideroblastic anemia. Alternatively spliced transcript variants encoding multiple isoforms hav
- 功能:
- 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.,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.,similarity:Belongs to the ABC transporter family. Heavy Metal importer (TC 3.A.1.210) subfamily.,similarity:Contains 1 ABC transmembrane type-1 domain.,similarity:Contains 1 ABC transporter domain.,subunit:Homodimer or heterodimer .,
- 细胞定位:
- Mitochondrion inner membrane ; Multi-pass membrane protein .
- 组织表达:
- Human esophagus tumor,Muscle,Placenta,Umbilical cord blood,
- Western Blot analysis of various cells using ABCB7 Polyclonal Antibody diluted at 1:1000
- Western Blot analysis of various cells using antibody diluted at 1:1000. Secondary antibody(catalog#:RS0002) was diluted at 1:20000
- Immunohistochemistry analysis of paraffin-embedded human thymus gland, using ABCB7 Antibody. The picture on the right is blocked with the synthesized peptide.
- Western blot analysis of the lysates from HUVECcells using ABCB7 antibody.