ABCB7 Antibody

CSB-PA575138

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Size:

100μl

US$297

Quantity:

Species Reactivity: Human

Raised in: Rabbit

Application: ELISA,WB,IHC

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Target Background

Product Details

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)

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.

Tested Applications

ELISA,WB,IHC

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.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

Protocols

ELISA Protocol Western Blotting(WB) Protocol Immunohistochemistry (IHC) Protocol

Datasheet & COA

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Images

Western blot analysis of extracts from HepG2 cells, using ABCB7 antibody.
Immunohistochemistry analysis of paraffin-embedded human thymus gland tissue using ABCB7 antibody.

Description

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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

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
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
findings support that ABCB7 is implicated in the phenotype of acquired RARS and suggest a relation between SF3B1 mutations and ABCB7 downregulation PMID:23070040
We describe a fourth family with X-linked sideroblastic anemia and ataxia and a novel mutation in the ABCB7 gene PMID:22398176
loss of the ABCB7 gene may be a pathogenetic factor underlying mitochondrial iron accumulation in RARS patients with idicXq13. PMID:21380928
ABCB7 may have a role in refractory anemia with ring sideroblasts PMID:18398482