產品編號 yb-1224R
英文名稱 Anti-ABCB6抗體
中文名稱 ATP結合蛋白家族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, 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.
Anti-ABCB6抗體 
說 明 書 0.1ml  0.2ml  
研究領域 腫瘤  心血管  信號轉導  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Guinea Pig, 
產品應用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 94kDa
細胞定位 細胞膜 線粒體
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human ABCB6
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed PubMed
產品介紹 background:
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.
蛋白對于紅細胞攜帶氧，肝細胞破壞毒素以及細胞從攝取營養物質后排放能量方面發揮重要的作用。