Signal Transduction Antibodies 1
Anti-ABCB7 Antibody (CAB14699)
- SKU:
- CAB14699
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Signal Transduction
Description
Antibody Name: | Anti-ABCB7 Antibody |
Antibody SKU: | CAB14699 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB IHC IF |
Reactivity: | Human, Mouse, Rat |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 503-753 of human ABCB7 (NP_004290.2). |
Application: | WB IHC IF |
Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
Reactivity: | Human, Mouse, Rat |
Positive Samples: | A-431, 293T, Mouse heart |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 503-753 of human ABCB7 (NP_004290.2). |
Purification Method: | Affinity purification |
Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Isotype: | IgG |
Sequence: | AIVG GSGS GKST IVRL LFRF YEPQ KGSI YLAG QNIQ DVSL ESLR RAVG VVPQ DAVL FHNT IYYN LLYG NISA SPEE VYAV AKLA GLHD AILR MPHG YDTQ VGER GLKL SGGE KQRV AIAR AILK DPPV ILYD EATS SLDS ITEE TILG AMKD VVKH RTSI FIAH RLST VVDA DEII VLDQ GKVA ERGT HHGL LANP HSIY SEMW HTQS SRVQ NHDN PKWE AKKE NISK EEER KKLQ EEIV NSVK GCGN CSC |
Gene ID: | 22 |
Uniprot: | O75027 |
Cellular Location: | Mitochondrion inner membrane, Multi-pass membrane protein |
Calculated MW: | 78kDa/82kDa |
Observed MW: | 83kDa |
Synonyms: | ABCB7, ABC7, ASAT, Atm1p, EST140535 |
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 have been observed for this gene. |
UniProt Protein Function: | 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. |
UniProt Protein Details: | Protein type:Transporter; Membrane protein, integral; Transporter, ABC family; Mitochondrial; Membrane protein, multi-pass Chromosomal Location of Human Ortholog: Xq13.3 Cellular Component: integral to membrane; mitochondrial inner membrane; mitochondrion Molecular Function:ATP binding; ATPase activity, coupled to transmembrane movement of substances; heme transporter activity Biological Process: cellular iron ion homeostasis; heme transport; transmembrane transport; transport Disease: Anemia, Sideroblastic, And Spinocerebellar Ataxia |
NCBI Summary: | 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 Code: | O75027 |
NCBI GenInfo Identifier: | 8928549 |
NCBI Gene ID: | 22 |
NCBI Accession: | O75027.2 |
UniProt Secondary Accession: | O75027,O75345, Q5VWY7, Q5VWY8, Q9BRE1, Q9UND1, Q9UP01 G3XAC4, |
UniProt Related Accession: | O75027 |
Molecular Weight: | 78,160 Da |
NCBI Full Name: | ATP-binding cassette sub-family B member 7, mitochondrial |
NCBI 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 |
UniProt Synonym Protein Names: | ATP-binding cassette transporter 7; ABC transporter 7 protein |
Protein Family: | ABC transporter B family |
UniProt Gene Name: | ABCB7 |
UniProt Entry Name: | ABCB7_HUMAN |
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