ABCB7 blocking peptide

ABCB7 Antibody (C-term) Blocking Peptide

• Gene Names: ABCB7; ABC7; ASAT; Atm1p; EST140535
• Synonyms: ABCB7; ABCB7 Antibody (C-term) Blocking Peptide; ATP-binding cassette sub-family B member 7; mitochondrial; ATP-binding cassette transporter 7; ABC transporter 7 protein; ABC7; ABCB7 blocking peptide

• Specificity: The synthetic peptide sequence used to generate the antibody was selected from the C-term region of human ABCB7. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
• Form/Format: The synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml deionized water for a final concentration of 1 mg/ml.
• Sequence Length: 753

• Cellular Location: Mitochondrion inner membrane; Multi-pass membrane protein
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for ABCB7 blocking peptide

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.

NCBI and Uniprot Product Information

• NCBI GI #: 42490749
• NCBI GeneID: 22
• NCBI Accession #: NP_004290
• NCBI GenBank Nucleotide #: NM_004299.4
• 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 1
• 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, ABC family; Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial; Transporter

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