Recombinant Human ABCB6/PRP protein is a Human Fragment protein, in the 598 to 818 aa range, expressed in Escherichia coli, with >95% purity and suitable for SDS-PAGE.
| Application | Reactivity | Dilution info | Notes |
|---|---|---|---|
Application SDS-PAGE | Reactivity Reacts | Dilution info - | Notes - |
ATP-dependent transporter that catalyzes the transport of a broad-spectrum of porphyrins from the cytoplasm to the extracellular space through the plasma membrane or into the vesicle lumen (PubMed:17661442, PubMed:23792964, PubMed:27507172, PubMed:33007128). May also function as an ATP-dependent importer of porphyrins from the cytoplasm into the mitochondria, in turn may participate in the de novo heme biosynthesis regulation and in the coordination of heme and iron homeostasis during phenylhydrazine stress (PubMed:10837493, PubMed:17006453, PubMed:23792964, PubMed:33007128). May also play a key role in the early steps of melanogenesis producing PMEL amyloid fibrils (PubMed:29940187). In vitro, it confers to cells a resistance to toxic metal such as arsenic and cadmium and against chemotherapeutics agent such as 5-fluorouracil, SN-38 and vincristin (PubMed:21266531, PubMed:25202056, PubMed:31053883). In addition may play a role in the transition metal homeostasis (By similarity).
MTABC3, PRP, UMAT, ABCB6, ATP-binding cassette sub-family B member 6, ABC-type heme transporter ABCB6, Mitochondrial ABC transporter 3, P-glycoprotein-related protein, Ubiquitously-expressed mammalian ABC half transporter, Mt-ABC transporter 3
Recombinant Human ABCB6/PRP protein is a Human Fragment protein, in the 598 to 818 aa range, expressed in Escherichia coli, with >95% purity and suitable for SDS-PAGE.
Constituents: 0.58% Sodium chloride, 0.32% Tris HCl
Purity is >95% by SDS-PAGE.
ATP-dependent transporter that catalyzes the transport of a broad-spectrum of porphyrins from the cytoplasm to the extracellular space through the plasma membrane or into the vesicle lumen (PubMed:17661442, PubMed:23792964, PubMed:27507172, PubMed:33007128). May also function as an ATP-dependent importer of porphyrins from the cytoplasm into the mitochondria, in turn may participate in the de novo heme biosynthesis regulation and in the coordination of heme and iron homeostasis during phenylhydrazine stress (PubMed:10837493, PubMed:17006453, PubMed:23792964, PubMed:33007128). May also play a key role in the early steps of melanogenesis producing PMEL amyloid fibrils (PubMed:29940187). In vitro, it confers to cells a resistance to toxic metal such as arsenic and cadmium and against chemotherapeutics agent such as 5-fluorouracil, SN-38 and vincristin (PubMed:21266531, PubMed:25202056, PubMed:31053883). In addition may play a role in the transition metal homeostasis (By similarity).
Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily.
N-glycosylated.
This product was previously labelled as ABCB6
ABCB6 also known as PRP is a transporter protein that plays important roles in cellular processes. This protein is part of the ATP-binding cassette (ABC) transporters specifically the B subfamily which are known for moving various molecules across membranes. ABCB6 has a molecular mass of approximately 92 kDa. It is expressed in various tissues including the liver and erythroid cells indicating its widespread importance in the body.
The ABCB6 protein is involved in the transport of porphyrins and other tetrapyrroles across membranes. These molecules are important for heme biosynthesis impacting red blood cell production and function. ABCB6 functions as a homodimer and is located on mitochondrial and lysosomal membranes. The transporter's ability to influence heme production links it to essential cellular metabolic processes.
The ABCB6 protein is engaged in the heme biosynthesis and mitochondrial function pathways. Its activity supports the synthesis of heme a fundamental component of hemoglobin and other heme-containing proteins. ABCB6 interacts with other proteins such as ALAS2 the erythroid-specific isoform of delta-aminolevulinic acid synthase regulating heme synthesis in erythroid cells. This connection highlights its role in maintaining adequate heme levels within cells.
ABCB6 has been implicated in conditions such as porphyria and anemia. Porphyria is a group of disorders stemming from defects in heme biosynthesis where mutations in ABCB6 may disrupt porphyrin transport aggravating symptoms. Anemia can also arise from impaired ABCB6 function as inadequate heme production affects red blood cell development. In these contexts ABCB6 can connect with proteins like ferrochelatase which plays a role in the final step of heme production illustrating the impact of ABCB6 on disease progression.
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