• Target：
• ABCB7

• Fields：
• >>ABC transporters

• Gene Name：
• ABCB7

• Protein Name：
• ATP-binding cassette sub-family B member 7 mitochondrial

• Human Gene Id：
• 22

• Human Swiss Prot No：
• O75027

• Mouse Swiss Prot No：
• Q61102

• Immunogen：
• The antiserum was produced against synthesized peptide derived from human ABCB7. AA range:691-740

• Specificity：
• ABCB7 Polyclonal Antibody detects endogenous levels of ABCB7 protein.

• Formulation：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• Source：
• Polyclonal, Rabbit,IgG

• Dilution：
• WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200

• Purification：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• Concentration：
• 1 mg/ml

• Storage Stability：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• Other Name：
• ABCB7;ABC7;ATP-binding cassette sub-family B member 7; mitochondrial;ATP-binding cassette transporter 7;ABC transporter 7 protein

• Observed Band(KD)：
• Human:83kD,Mouse/Rat 100kD

• Background：
• 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

• Function：
• 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 .,

• Subcellular Location：
• Mitochondrion inner membrane ; Multi-pass membrane protein .

• Expression：
• Human esophagus tumor,Muscle,Placenta,Umbilical cord blood,

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