Rabbit ABCB6 Polyclonal Antibody | anti-ABCB6 antibody

ABCB6 Antibody

Cat. Num. AAA9611556

• Gene Names: ABCB6; ABC; LAN; PRP; umat; MTABC3
• Reactivity: Human; Predicted: Pig, Bovine, Horse, Sheep, Rabbit, Dog, Chicken
• Applications: Western Blot
• Purity: The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin (Thermo Fisher Scientific).
• Synonyms: ABCB6; Polyclonal Antibody; ABCB6 Antibody; ABC; ABC transporter umat; ABC14; Abcb6; ABCB6_HUMAN; ATP binding cassette sub family B member 6; mitochondrial precursor; ATP binding cassette; sub family B (MDR/TAP); member 6; ATP-binding cassette half-transporter; ATP-binding cassette sub-family B member 6; mitochondrial; DUH3; LAN; MCOPCB7; mitochondrial ABC transporter 3; Mt ABC transporter 3; Mt-ABC transporter 3; MTABC3; P glycoprotein related protein; P-glycoprotein-related protein; PRP; Ubiquitously expressed mammalian ABC half transporter; Ubiquitously-expressed mammalian ABC half transporter; UMAT; anti-ABCB6 antibody

• Host: Rabbit
• Reactivity: Human; Predicted: Pig, Bovine, Horse, Sheep, Rabbit, Dog, Chicken
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: ABCB6 Antibody detects endogenous levels of ABCB6.
• Purity/Purification: The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin (Thermo Fisher Scientific).
• Form/Format: Liquid; Phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1mg/ml (varies by lot)
• Sequence Length: 842

• Applicable Applications for anti-ABCB6 antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:2000
• Immunogen: A synthesized peptide derived from human ABCB6.
• Tissue Specificity: Widely expressed. High expression is detected in the retinal epithelium.
• Fragment: Fab Fragment
• Conjugate: Unconjugated
• Subcellular Location: Endosome; Endoplasmic Reticulum; Mitochondrion; Golgi Apparatus; Plasma Membrane
• Preparation and Storage: Store at -20 degree C. Stable for 12 months from date of receipt.

Western Blot (WB)

(Western blot analysis of extracts from Raji cells, using ABCB6 Antibody. The lane on the left was treated with blocking peptide.)

Related Product Information for anti-ABCB6 antibody

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

Subunit Structure: Homodimer.

Similarity: Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily.

NCBI and Uniprot Product Information

• NCBI GI #: 13123949
• NCBI GeneID: 10058
• UniProt Accession #: Q9NP58
• Molecular Weight: Observed: 84 kDa; Predicted: 94 kDa
• NCBI Official Full Name: ATP-binding cassette sub-family B member 6, mitochondrial
• NCBI Official Synonym Full Names: ATP binding cassette subfamily B member 6 (Langereis blood group)
• NCBI Official Symbol: ABCB6
• NCBI Official Synonym Symbols: ABC; LAN; PRP; umat; MTABC3
• NCBI Protein Information: ATP-binding cassette sub-family B member 6, mitochondrial
• UniProt Protein Name: ATP-binding cassette sub-family B member 6, mitochondrial
• Protein Family: ABC transporter B family
• UniProt Gene Name: ABCB6
• UniProt Synonym Gene Names: MTABC3; PRP; UMAT; Mt-ABC transporter 3
• UniProt Entry Name: ABCB6_HUMAN

NCBI Description

This gene encodes a member of the ATP-binding cassette (ABC) transporter superfamily. ABC proteins transport various molecules across extra- and intra-cellular membranes. This protein is a member of the heavy metal importer subfamily and plays a role in porphyrin transport. This gene is the molecular basis of the Langereis (Lan) blood group antigen and mutations in this gene underlie familial pseudohyperkalemia and dyschromatosis universalis hereditaria. [provided by RefSeq, Mar 2017]

Uniprot Description

ABCB6: Binds heme and porphyrins and functions in their ATP- dependent uptake into the mitochondria. Plays a crucial role in heme synthesis. Defects in ABCB6 are the cause of microphthalmia, isolated, with coloboma, type 7 (MCOPCB7). A disorder of eye formation, ranging from small size of a single eye to complete bilateral absence of ocular tissues. Ocular abnormalities like opacities of the cornea and lens, scaring of the retina and choroid, cataract and other abnormalities like cataract may also be present. Ocular colobomas are a set of malformations resulting from abnormal morphogenesis of the optic cup and stalk, and the fusion of the fetal fissure (optic fissure). Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Transporter, ABC family; Hydrolase; Mitochondrial; Membrane protein, integral; Transporter; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 2q36

Cellular Component: mitochondrial envelope; Golgi apparatus; mitochondrial outer membrane; mitochondrion; endoplasmic reticulum; plasma membrane; ATP-binding cassette (ABC) transporter complex; endosome; integral to mitochondrial outer membrane

Molecular Function: efflux transmembrane transporter activity; heme-transporting ATPase activity; heme transporter activity; heme binding; ATP binding

Biological Process: skin development; heme transport; cellular iron ion homeostasis; transport; brain development; porphyrin biosynthetic process; transmembrane transport

Disease: Dyschromatosis Universalis Hereditaria 3; Blood Group, Langereis System; Microphthalmia, Isolated, With Coloboma 7