造血干细胞抗原颁顿133抗体
规格：1尘驳/1尘濒
英文名： CD133
别名： AC133; Antigen AC133; Hematopoietic stem cell antigen; hProminin; PROM1; Prominin I; Prominin like protein 1 precursor; Prominin mouse like 1; prominin1; PROML1; CD133; CORD12; MCDR2; MSTP061; PROML1;
分子量： 95kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human CD133
交叉反应：Human, Mouse, Rat,
造血干细胞抗原颁顿133抗体细胞定位：细胞膜
产物介绍：background: This gene encodes a pentaspan transmembrane glycoprotein. The protein localizes to membrane protrusions and is often expressed on 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] Function: Binds cholesterol in cholesterol-containing plasma membrane microdomains. Proposed to play a role in apical plasma membrane organization of epithelial cells. During early retinal development acts as a key regulator of disk morphogenesis. Involved in regulation of MAPK and Akt signaling pathways. In neuroblastoma cells suppresses cell differentiation such as neurite outgrowth in a RET-dependent manner. Subunit: Interacts with CDHR1 and with actin filaments. Subcellular Location: Cell projection, cilium, photoreceptor outer segment. Isoform 1: Apical cell membrane; Multi-pass membrane protein. Cell projection, microvillus membrane; Multi-pass membrane protein. Note=Found in extracellular membrane particles in various body fluids such as cerebrospinal fluid, saliva, seminal fluid and urine. Tissue Specificity: Isoform 1 is selectively expressed on CD34 hematopoietic stem and progenitor cells in and fetal bone marrow, fetal liver, cord blood and peripheral blood. Isoform 1 is not detected on other blood cells. Isoform 1 is 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. Isoform 2 is predominantly expressed in fetal liver, skeletal muscle, kidney, and heart as well as pancreas, kidney, liver, lung, and placenta. Isoform 2 is highly expressed in fetal liver, low in bone marrow, and barely detectable in peripheral blood. Isoform 2 is expressed on hematopoietic stem cells and in epidermal basal cells (at protein level). Expressed in retina by rod and cone photoreceptor cells (at protein level). Post-translational modifications造血干细胞抗原颁顿133抗体: Isoform 1 and isoform 2 are glycosylated. 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. 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. 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. 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. Similarity: Belongs to the prominin family. Gene ID: 8842 Database links: Entrez Gene: 8842 Human
Entrez Gene: 19126 Mouse
SwissProt: O43490 Human
SwissProt: O54990 Mouse
Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 干细胞标志物 一般认为，VEGFR2（血管内皮生长因子受体2）是HSCs（造血干细胞）的特异性的表面标志。近来经研究发现CD133分子是HSCs（造血干细胞）特异性标志。CD133即AC133，是一个新发现的HSCs（造血干细胞）表面标志，在HSCs（造血干细胞）分化成熟过程中，造血干细胞抗原颁顿133抗体CD133的含量迅速降低。EPCs(血管内皮前体细胞)区别于成熟内皮细胞的主要标志是CD133。 经研究发现内皮细胞不能结合CD133的抗体。证实分化成熟的内皮细胞不具有CD133。这些说明CD133可以作为EPCs(血管内皮前体细胞)区别于成熟内皮细胞的一个表面标志.
造血干细胞抗原颁顿133抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 **学 干细胞
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid