CD133 (PN0463) Nb-FC recombinant antibody
- 货号：YA0071
- 应用：FCM;ELISA
- 种属：Human
- 简介：
- >>Transcriptional misregulation in cancer
- 免疫原：
- Purified recombinant Human CD133
- 特异性：
- This recombinant monoclonal antibody can detects endogenous levels of CD133 protein.
- 组成：
- Phosphate-buffered solution
- 来源：
- Camel, chimeric fusion of Nanobody (VHH) and mouse IgG1 Fc domain , recombinantly produced from 293F cell
- 稀释：
- ELISA 1:5000-100000;FCM 1-2μg/Test
- 纯化工艺：
- Recombinant Expression and Affinity purified
- 浓度：
- Please check the information on the tube
- 储存：
- -15°C to -25°C/1 year(Avoid freeze / thaw cycles)
- 其他名称：
- PROM1;Prominin-1;Antigen AC133;Prominin-like protein 1;CD133
- 背景：
- 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],
- 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
- 细胞定位：
- Apical cell membrane ; Multi-pass membrane protein . Cell projection, microvillus membrane ; Multi-pass membrane protein . Cell projection, cilium, photoreceptor outer segment . Endoplasmic reticulum. Endoplasmic reticulum-Golgi intermediate compartment. Found in extracellular membrane particles in various body fluids such as cerebrospinal fluid, saliva, seminal fluid and urine.
- 组织表达：
- Isoform 1 is selectively expressed on CD34 hematopoietic stem and progenitor cells in adult and fetal bone marrow, fetal liver, cord blood and adult 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 adult 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 3 is expressed on hematopoietic stem cells and in epidermal basal cells (at protein level). Expressed in adult retina by rod and
