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ABCB6 Rabbit pAb (bs-1224R)  
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產(chǎn)品編號(hào) bs-1224R
英文名稱 ABCB6 Rabbit pAb
中文名稱 ATP結(jié)合蛋白家族6抗體
別    名 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,  
研究領(lǐng)域 腫瘤  心血管  信號(hào)轉(zhuǎn)導(dǎo)  新陳代謝  線粒體  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human,Mouse,Rat (predicted: Pig,Cow,Dog,GuineaPig,Horse)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 94 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞膜 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ABCB6: 401-520/842 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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

Entrez Gene: 140669 Rat

Omim: 605452 Human

SwissProt: Q9NP58 Human

SwissProt: Q9DC29 Mouse

SwissProt: O70595 Rat

SwissProt: Q9Z1S2 Rat

Unigene: 107911 Human

Unigene: 28663 Mouse

Unigene: 54408 Rat



ABCB6蛋白對(duì)于紅細(xì)胞攜帶氧,肝細(xì)胞破壞毒素以及細(xì)胞從攝取營(yíng)養(yǎng)物質(zhì)后排放能量方面發(fā)揮重要的作用。
產(chǎn)品圖片
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 (bs-1224R) 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 (bs-1224R) 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(bs-1224R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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