CD133 (1T14) Rabbit Monoclonal Antibody
产品基本信息
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产品货号
RM8259
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别名
PROM1;Prominin-1;Antigen AC133;Prominin-like protein 1;CD133
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产品名称
CD133 (1T14) Rabbit Monoclonal Antibody
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类别
抗原抗体
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基因名称
PROM1
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蛋白名称
Prominin-1
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Clonality
Monoclonal
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推荐应用
WB,IHC-P,IF-P,IF-F,IF-ICC,IP,ELISA
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反应种属
Human
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存储缓冲液
PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
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Human Gene ID
8842
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Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=8842
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Human Swissprot No.
O43490
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Human Swissprot Link
http://www.uniprot.org/uniprotkb/O43490/entry
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Mouse Swissprot No.
O54990
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Mouse Swissprot Link
http://www.uniprot.org/uniprot/O54990
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稀释度
IHC-P 1:20-100,WB 1:1000-5000,IF-P/IF-F/IF-ICC 1:200-1000,ELISA 1:5000-20000,IP 1:50-200
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参考分子量
133kDa
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预测分子量
97kDa
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运输及保存条件
-20°C/1 year(Do not lower than -25°C)
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宿主
Rabbit
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同种型
Monoclonal, rabbit, IgG, Kappa
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背景介绍
This gene encodes a pentaspan transmembrane glycoprotein. The protein localizes to membrane protrusions and is often expressed on adult stem cells, where it is thought to function in maintaining stem cell properties by suppressing differentiation. Mutations in this gene have been shown to result in retinitis pigmentosa and Stargardt disease. Expression of this gene is also associated with several types of cancer. This gene is expressed from at least five alternative promoters that are expressed in a tissue-dependent manner. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2009],
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功能
disease:Defects in PROM1 are the cause of cone-rod dystrophy type 12 (CORD12) [MIM:612657]. CORD12 is an inherited retinal dystrophy characterized by retinal pigment deposits visible on fundus examination, predominantly in the macular region, and initial loss of cone photoreceptors followed by rod degeneration. This leads to decreased visual acuity and sensitivity in the central visual field, followed by loss of peripheral vision. Severe loss of vision occurs earlier than in retinitis pigmentosa.,disease:Defects in PROM1 are the cause of retinal macular dystrophy type 2 (MCDR2) [MIM:608051]. MCDR2 is a bull's-eye macular dystrophy characterized by bilateral annular atrophy of retinal pigment epithelium at the macula.,disease:Defects in PROM1 are the cause of retinitis pigmentosa type 41 (RP41) [MIM:612095]; also known as retinal degeneration autosomal recessive prominin-related. RP is a retinal dystrophy belonging to the group of pigmentary retinopathies. RP is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.,disease:Defects in PROM1 are the cause of Stargardt disease type 4 (STGD4) [MIM:603786]. Stargardt disease is the most common hereditary macular degeneration. It is characterized by decreased central vision, atrophy of the macula and underlying retinal pigment epithelium, and frequent presence of prominent flecks in the posterior pole of the retina.,online information:Retina International's Scientific Newsletter,PTM:Glycosylated.,similarity:Belongs to the prominin family.,subunit:Interacts with PCDH21 and with actin filaments.,tissue specificity:Selectively expressed on CD34 hematopoietic stem and progenitor cells in adult and fetal bone marrow, fetal liver, cord blood and adult peripheral blood. Not detected on other blood cells. Also expressed in a number of non-lymphoid tissues including retina, pancreas, placenta, kidney, liver, lung, brain and heart. Found in saliva within small membrane particles.,
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期货
现货
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纯化
Protein A
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