Rabbit anti-Human ABCB7 Polyclonal Antibody | anti-ABCB7 antibody

ABCB7 Antibody

Cat. Num. AAA9415210

• Gene Names: ABCB7; ABC7; ASAT; Atm1p; EST140535
• Reactivity: Human
• Applications: Immunohistochemistry
• Purity: Antigen Affinity Purification
• Synonyms: ABCB7; Polyclonal Antibody; ABCB7 Antibody; ABC7; ASAT; Atm1p; EST140535; anti-ABCB7 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous levels of total ABCB7 protein.
• Purity/Purification: Antigen Affinity Purification
• Form/Format: Rabbit IgG in pH7.4 PBS, 0.05% NaN3, 40% Glycerol.
• Concentration: 1.1 mg/ml (varies by lot)
• Sequence Length: 752

• Applicable Applications for anti-ABCB7 antibody: Immunohistochemistry (IHC)
• Application Notes: Immunohistochemistry: 1:25-1:100
• Immunogen: Fusion protein of human ABCB7
• Immunogen Type: Protein
• Preparation and Storage: Store at -20 degree C

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded Human esophagus cancer tissue using at dilution 1/20.)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded Human thyroid cancer tissue using at dilution 1/20.)

Related Product Information for anti-ABCB7 antibody

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 isoformshave been observed for this gene.

Product Categories/Family for anti-ABCB7 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 411147363
• NCBI GeneID: 22
• NCBI Accession #: NP_001258625.1
• NCBI GenBank Nucleotide #: NM_001271696.1
• UniProt Accession #: O75027; O75345; Q5VWY7; Q5VWY8; Q9BRE1; Q9UND1; Q9UP01; G3XAC4
• Molecular Weight: 78,160 Da
• 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

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

Chromosomal Location of Human Ortholog: Xq13.3

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

Molecular Function: heme transporter activity

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

Disease: Anemia, Sideroblastic, And Spinocerebellar Ataxia