• ABCB7 Polyclonal Antibody
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Upingbio

YP-Ab-00652-100UL

ABCB7 Polyclonal Antibody

ABCB7 Polyclonal Antibody

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  • Gene Name: ABCB7
  • 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.
  • Composition: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • Source: Polyclonal, Rabbit,IgG
  • Dilution ratio: WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/40000.. IF 1:50-200
  • Purification process: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • Concentration: 1 mg/ml
  • Storage: -20°C/1 year
  • Other Names: ABCB7; ABC7; ATP-binding cassette sub-family B member 7; mitochondrial; ATP-binding cassette transporter 7; ABC transporter 7 protein
  • 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 .,
  • Species: Human;Rat;Mouse;
  • Range: WB;IHC;IF;ELISA
  • Protein: ATP-binding cassette sub-family B member 7 mitochondrial
  • DA: Human:83kD,Mouse/Rat 100kD
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