PROM1 | CD133 Recombinant Rabbit mAb (一抗) - WB,IHC-P,IHC-F,IF | Bioss

bsm-62477R

重组兔单抗、mIHC精品抗体

PROM1 | CD133 Recombinant Rabbit mAb

造血干细胞抗原CD133重组兔单抗

AC133; CD133; CORD12; MCDR2; MSTP061; PROML1; RP41; STGD4; 4932416E19Rik; Prom; Prom-1; PROM1_HUMAN; PROM1; Antigen AC133; Prominin-like protein 1; PROM1_MOUSE; Antigen AC133 homolog; prominin 1; prominin (mouse)-like 1; macular dystrophy, retinal 2; Stargardt disease 4 (autosomal dominant); AC133 antigen; Retinitis pigmentosa 41, Cone-rod dystrophy 12

Rabbit

A synthesized peptide derived from human CD133: 250-380

IgG

Liquid

affinity purified by Protein A

Recombinant

11G1

97 kDa

130 kDa

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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，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.

May play a role in cell differentiation, proliferation and apoptosis. Binds cholesterol in cholesterol-containing plasma membrane microdomains and may play a role in the organization of the apical plasma membrane in 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.

Prom1

Prominin-1

Interacts with CDHR1 and with actin filaments.

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.

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 2 is expressed on hematopoietic stem cells and in epidermal basal cells (at protein level). Expressed in adult retina by rod and cone photoreceptor cells (at protein level).

Isoform 1 and isoform 2 are glycosylated.

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.

Belongs to the prominin family.

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.