ABCB7 Antibody

Code CSB-PA575138
Size US$297 How to order?
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  • Western blot analysis of extracts from HepG2 cells, using ABCB7 antibody.
  • Immunohistochemistry analysis of paraffin-embedded human thymus gland tissue using ABCB7 antibody.
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Product Details

Full Product Name Rabbit anti-Homo sapiens (Human) ABCB7 Polyclonal antibody
Uniprot No. O75027
Target Names ABCB7
Alternative Names ABC transporter 7 protein antibody; ABC7 antibody; Abcb7 antibody; ABCB7_HUMAN antibody; ASAT antibody; Atm1p antibody; ATP binding cassette 7 antibody; ATP binding cassette sub family B (MDR/TAP) member 7 antibody; ATP binding cassette sub family B member 7 antibody; ATP binding cassette sub family B member 7 mitochondrial antibody; ATP binding cassette transporter 7 antibody; ATP-binding cassette sub-family B member 7 antibody; ATP-binding cassette transporter 7 antibody; EST140535 antibody; MDR7 antibody; mitochondrial antibody; Multidrug resistance protein 7 antibody; P-glycoprotein 7 antibody; PGP7 antibody
Raised in Rabbit
Species Reactivity Human
Immunogen Synthesized peptide derived from internal of Human ABCB7.
Immunogen Species Homo sapiens (Human)
Clonality Polyclonal
Purification Method The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration It differs from different batches. Please contact us to confirm it.
Form Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Tested Applications ELISA,WB,IHC
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:3000
IHC 1:50-1:100
Protocols ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol
Troubleshooting and FAQs Antibody FAQs
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Exports glutathione-coordinated iron-sulfur clusters such as [2Fe-2S]-(GS)4 cluster from the mitochondria to the cytosol in an ATP-dependent manner allowing the assembly of the cytosolic iron-sulfur (Fe/S) cluster-containing proteins and participates in iron homeostasis. Moreover, through a functional complex formed of ABCB7, FECH and ABCB10, also plays a role in the cellular iron homeostasis, mitochondrial function and heme biosynthesis. In cardiomyocytes, regulates cellular iron homeostasis and cellular reactive oxygen species (ROS) levels through its interaction with COX4I1. May also play a role in hematopoiesis.
Gene References into Functions
  1. data support a model in which cycloheximide -induced downregulation of the iron exporter ABCB7 mRNA transcript resulting from aberrant splicing caused by mutant SF3B1 underlies the increased mitochondrial iron accumulation found in MDS patients with ring sideroblasts PMID: 27211273
  2. A missense mutation in the ABCB7 is a major causative factor of the cerebellar hypoplasia/atrophy found in affected individuals of a Buryat family who had no evidence of sideroblastic anemia. PMID: 26242992
  3. findings support that ABCB7 is implicated in the phenotype of acquired RARS and suggest a relation between SF3B1 mutations and ABCB7 downregulation PMID: 23070040
  4. We describe a fourth family with X-linked sideroblastic anemia and ataxia and a novel mutation in the ABCB7 gene PMID: 22398176
  5. loss of the ABCB7 gene may be a pathogenetic factor underlying mitochondrial iron accumulation in RARS patients with idicXq13. PMID: 21380928
  6. ABCB7 may have a role in refractory anemia with ring sideroblasts PMID: 18398482

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Involvement in disease Anemia, sideroblastic, spinocerebellar ataxia (ASAT)
Subcellular Location Mitochondrion inner membrane; Multi-pass membrane protein.
Protein Families ABC transporter superfamily, ABCB family, Heavy Metal importer (TC 3.A.1.210) subfamily
Database Links

HGNC: 48

OMIM: 300135

KEGG: hsa:22

STRING: 9606.ENSP00000253577

UniGene: Hs.370480

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