Rabbit ABCB7 Polyclonal Antibody | anti-ABCB7 antibody

ABCB7 Polyclonal Antibody

Cat. Num. AAA3005652

• Gene Names: ABCB7; ABC7; ASAT; Atm1p; EST140535
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: ABCB7; Polyclonal Antibody; ABCB7 Polyclonal Antibody; ATP-binding cassette sub-family B member 7; mitochondrial; ATP-binding cassette transporter 7; ABC transporter 7 protein; ABC7; ABCB-7; anti-ABCB7 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: ABCB7 polyclonal antibody detects endogenous levels of ABCB7 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 752

• Applicable Applications for anti-ABCB7 antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:1000
• Immunogen: Synthetic peptide, corresponding to amino acids 707-752 of Human ABCB7.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of ABCB7 polyclonal antibody at 1:500 dilutionLane1:Hela cell lysateLane2:Raw264. 7 cell lysateLane3:PC12 cell lysate)

Related Product Information for anti-ABCB7 antibody

The peroxisomal membrane contains several ATP-binding cassette (ABC) transporters, ABCD1-4 that are known to be present in the human peroxisome membrane. All four proteins are ABC half-transporters, which dimerize to form an active transporter. A mutation in the ABCD1 causes X-linked adrenoleukodystrophy (X-ALD), a peroxisomal disorder which affects lipid storage. ABCD2 in mouse, is expressed at high levels in the brain and adrenal organs, which are adversely affected in X-ALD. The peroxisomal membrane comprises 2 quantitatively major proteins, PMP22 and ABCD3. ABCD3 is associated with irregularly shaped vesicles which may be defective peroxisomes or peroxisome precursors. ABCD4 localizes to peroxisomes. ABCB7 is a half-transporter involved in the transport of heme from the mitochondria to the cytosol and maps to human chromosome Xq13.1-q13.3.

NCBI and Uniprot Product Information

• NCBI GI #: 411147363
• NCBI GeneID: 22
• NCBI Accession #: NP_001258625.1
• NCBI GenBank Nucleotide #: NP_001258625.1
• UniProt Accession #: O75027
• Molecular Weight: ~ 95kDa
• NCBI Official Full Name: ATP-binding cassette sub-family B member 7, mitochondrial isoform 2
• NCBI Official Synonym Full Names: ATP binding cassette subfamily B member 7
• NCBI Official Symbol: ABCB7
• NCBI Official Synonym Symbols: ABC7; ASAT; Atm1p; EST140535
• NCBI Protein Information: ATP-binding cassette sub-family B member 7, mitochondrial
• UniProt Protein Name: ATP-binding cassette sub-family B member 7, mitochondrial
• Protein Family: ABC transporter B family
• UniProt Gene Name: ABCB7
• UniProt Synonym Gene Names: ABC7; ABC transporter 7 protein
• UniProt Entry Name: ABCB7_HUMAN

NCBI Description

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]

Uniprot Description

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