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Product Name ABCB6 Chinese Name ATPBinding protein家族6抗体 Alias ABC; ABC14; EST45597; ABC 14; ABC-14; ABCB 6; ABCB-6; PRP; UMAT; ABC transporter umat; MTABC3; Mt-ABC transporter 3; umat; Mitochondrial precursor; Mitochondrial ABC transporter 3; Mitochondrial abc protein 3; ATP-binding cassette, sub-family B, member 6, mitochondrial precursor; ATP binding cassette, sub family B (MDR/TAP), member 6; P glycoprotein related protein; Abcb6; ABCB6_HUMAN; ATP binding cassette sub family B member 6, mitochondrial precursor; ATP-binding cassette sub-family B member 6; Mitochondrial ABC transporter 3; mitochondrial; Mt ABC transporter 3; P-glycoprotein-related protein; Ubiquitously-expressed mammalian ABC half transporter; UMAT; Ubiquitously expressed mammalian ABC half transporter. Research Area Tumour Cardiovascular Signal transduction The new supersedes the old Mitochondrion Immunogen Species Rabbit Clonality Polyclonal React Species Human, Mouse, Rat, (predicted: Dog, Pig, Cow, Horse, Guinea Pig, ) Applications ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 94kDa Cellular localization The cell membrane Mitochondrion Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human ABCB6: 401-520/842 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail The membrane-associated protein encoded by this gene 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 half-transporter likely plays a role in mitochondrial function. Localized to 2q26, this gene is considered a candidate gene for lethal neonatal metabolic syndrome, a disorder of mitochondrial function.
Function:
Binds heme and porphyrins and functions in their ATP-dependent uptake into the mitochondria. Plays a crucial role in heme synthesis
Subunit:
Homodimer.
Subcellular Location:
Cell membrane. Mitochondrion outer membrane; Multi-pass membrane protein. Endoplasmic reticulum. Golgi apparatus.
Tissue Specificity:
Widely expressed. High expression is detected in the retinal epithelium.
DISEASE:
Microphthalmia, isolated, with coloboma, 7 (MCOPCB7) [MIM:614497]: 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). Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily.
Contains 1 ABC transmembrane type-1 domain.
Contains 1 ABC transporter domain.
SWISS:
Q9NP58
Gene ID:
10058
Database links:Entrez Gene: 10058 Human
Entrez Gene: 74104 Mouse
Omim: 605452 Human
SwissProt: Q9NP58 Human
SwissProt: Q9DC29 Mouse
Unigene: 107911 Human
Unigene: 28663 Mouse
Unigene: 54408 Rat
ABCB6蛋白对于红细胞携带氧,肝细胞破坏毒素以及细胞从摄取营养物质后排放能量方面发挥重要的作用。Product Picture Paraformaldehyde-fixed, paraffin embedded (rat kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ABCB6) Polyclonal Antibody, Unconjugated (SL1224R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.Paraformaldehyde-fixed, paraffin embedded (rat liver); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ABCB6) Polyclonal Antibody, Unconjugated (SL1224R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.Tissue/cell: rat liver tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-ABCB6 Polyclonal Antibody, Unconjugated(SL1224R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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