ABCB6 antibody

Este producto es parte de ABCB - ATP binding cassette subfamily B member
ABCB6 antibody
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Name
ABCB6 antibody
Category
Primary Antibodies
Provider
FineTest
Reference
FNab00032
Tested Applications
ELISA, WB, IHC,IF

Description

Binds heme and porphyrins and functions in their ATP-dependent uptake into the mitochondria. Plays a crucial role in heme synthesis.

Documentos del producto

Instrucciones
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Data sheet
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Especificaciones del producto

Category
Primary Antibodies
Immunogen Target
ATP-binding cassette, sub-family B(MDR/TAP), member 6 (ABCB6)
Host
Rabbit
Reactivity
Human
Recommended Dilution
WB: 1:500-1:5000; IHC: 1:20-1:200; IF: 1:20-1:200
Clonality
polyclonal
Conjugation
Unconjugated
Isotype
IgG
Observed MW
94 kDa
Purity
≥95% as determined by SDS-PAGE
Purification
Immunogen affinity purified
Size 1
100µg
Form
liquid
Tested Applications
ELISA, WB, IHC,IF
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20℃ for 12 months(Avoid repeated freeze / thaw cycles.)
UniProt ID
Q9NP58
Gene ID
10058
Alias
LAN,MTABC3
Background
Antibody anti-ABCB6
Status
RUO
Note
Mol. Weight 94 kDa

Background

ABCB6 is a mitochondrial ATP-binding cassette transporter critical for the transport of porphyrins and precursors involved in heme biosynthesis. Localized to the outer mitochondrial membrane, ABCB6 facilitates the uptake of coproporphyrinogen III, a precursor in the heme biosynthesis pathway, enabling its conversion to protoporphyrin IX within mitochondria. ABCB6 is highly expressed in erythroid cells, liver, and other tissues with high heme demands, supporting processes such as oxygen transport and detoxification. In addition to its role in heme production, ABCB6 is involved in the cellular response to oxidative stress and drug metabolism. Mutations in ABCB6 have been associated with disorders like familial pseudohyperkalemia and dyschromatosis universalis hereditaria, affecting red blood cells and pigmentation, respectively. Dysregulation of ABCB6 impacts mitochondrial function and porphyrin transport, contributing to metabolic disorders and emphasizing its importance in maintaining cellular and systemic homeostasis.

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