anti- ABCB7 antibody

Este producto es parte de ABCB - ATP binding cassette subfamily B member
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935106861
info@markelab.com
name
anti- ABCB7 antibody
category
Primary Antibodies
provider
FineTest
reference
FNab00033
tested applications
ELISA, WB, IP

Description

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.

Documents del producto

Instrucciones
Descargar
Data sheet

Product specifications

Category
Primary Antibodies
Immunogen Target
ATP-binding cassette, sub-family B(MDR/TAP), member 7
Host
Rabbit
Reactivity
human,mouse,rat
Recommended Dilution
WB: 1:500-1:1000; IP: 1:500-1:1000
Clonality
polyclonal
Conjugation
Unconjugated
Isotype
IgG
Observed MW
83 kDa
Purity
≥95% as determined by SDS-PAGE
Purification
Immunogen affinity purified
Size 1
100µg
Form
liquid
Tested Applications
ELISA, WB, IP
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3,-20℃ for 12 months(Avoid repeated freeze / thaw cycles.)
UniProt ID
O75027
Gene ID
22
Alias
ABC7,ASAT,Atm1p,EST140535
Background
Antibody anti-ABCB7
Status
RUO
Note
This product is for research use only.

Descripción

ABCB7 is a mitochondrial transporter essential for the biogenesis of iron-sulfur clusters, which are critical cofactors for enzymes involved in cellular respiration, DNA repair, and metabolic processes. ABCB7 facilitates the export of iron-sulfur cluster intermediates from mitochondria to the cytosol, a key step in ensuring proper assembly and function of cytosolic and nuclear iron-sulfur proteins. Highly expressed in metabolically active tissues, such as the liver, bone marrow, and red blood cells, ABCB7 plays a vital role in maintaining iron homeostasis and mitochondrial function. Mutations in the ABCB7 gene are linked to X-linked sideroblastic anemia with ataxia, characterized by defective hemoglobin synthesis, neurological impairments, and mitochondrial dysfunction. Additionally, ABCB7 has been implicated in oxidative stress responses, highlighting its broader role in cellular protection and energy regulation. Its functions in iron-sulfur cluster assembly and export underscore its critical importance in mitochondrial and cellular health.

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The membrane-associated protein ABCB7 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 implicated in X-linked sideroblastic anemia with ataxia.

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