ABCB7, Polyclonal Antibody

Anti-ABCB7 Antibody (aa691-740) IHC-plus; ABCB7; ABC transporter 7 protein; ABC7; Atm1p; ATP-binding cassette 7; ASAT; EST140535; Human ABCB7

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

ABCB7 Antibody detects endogenous levels of total ABCB7 protein.

Immunoaffinity Purified

PBS (without Mg2+, Ca2+), pH 7.4, 150 mM sodium chloride, 0.02% sodium azide, 50% glycerol

1 mg/ml (lot specific)

Short term 4 degree C, long term aliquot and store at -20 degree C, avoid freeze thaw cycles.

Small volumes of anti-ABCB7 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Subfamily:-ATP-binding-cassette---ABCB/MDR">Family: Transporter
Subfamily: ATP-binding cassette - ABCB/MDR

Immunohistochemistry (IHC - Paraffin), ELISA (EIA)

ELISA (1:5000), IHC-P (5 ug/ml)

Anti-ABCB7 antibody IHC of human lung, alveoli. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.

78,160 Da

ATP-binding cassette, sub-family B (MDR/TAP), member 7

ATP-binding cassette sub-family B member 7, mitochondrial; ABC transporter 7 protein; ATP-binding cassette transporter 7

ATP-binding cassette sub-family B member 7, mitochondrial

ABC7; ABC transporter 7 protein  [Similar Products]

ABCB7_HUMAN

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 have been observed for this gene. [provided by RefSeq, Nov 2012]

ABCB7: 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. Defects in ABCB7 are the cause of X-linked sideroblastic anemia with ataxia (ASAT). ASAT is a recessive disorder characterized by an infantile to early childhood onset of nonprogressive cerebellar ataxia and mild anemia with hypochromia and microcytosis. Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Transporter; Transporter, ABC family; Membrane protein, multi-pass; Membrane protein, integral; Mitochondrial

Chromosomal Location of Human Ortholog: Xq13.3

Cellular Component: mitochondrion; mitochondrial inner membrane; integral to membrane

Molecular Function: ATPase activity, coupled to transmembrane movement of substances; heme transporter activity; ATP binding

Biological Process: heme transport; transport; cellular iron ion homeostasis; transmembrane transport

Disease: Anemia, Sideroblastic, And Spinocerebellar Ataxia

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