产品名称
ABCB7 Rabbit Polyclonal Antibody
别名
ABCB7; ABC7; ATP-binding cassette sub-family B member 7; mitochondrial; ATP-binding cassette transporter 7; ABC transporter 7 protein
蛋白名称
ATP-binding cassette sub-family B member 7 mitochondrial
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=22
Human Swissprot No.
O75027
Human Swissprot Link
http://www.uniprot.org/uniprotkb/O75027/entry
Mouse Swissprot No.
Q61102
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q61102
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.
稀释度
WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200
预测分子量
Human:83kD,Mouse/Rat 100kD
宿主
Polyclonal, Rabbit,IgG
背景介绍
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
组织表达
Human esophagus tumor,Muscle,Placenta,Umbilical cord blood,
细胞定位
Mitochondrion inner membrane ; Multi-pass membrane protein .
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 .,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.