产品名称
Anti-PDGFRB (Ab-751) antibody produced in rabbit, affinity isolated antibody
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
~190 kDa
species reactivity
rat, human
concentration
1 mg/mL
technique(s)
western blot: 1:500-1:1000
isotype
IgG
immunogen sequence
(V-D-Y-V-P)
NCBI accession no.
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... PDGFRB(5159)
Biochem/physiol Actions
PDGFRB (platelet derived growth factor receptor β), upon interaction with PGDFB, regulates multiple processes such as, sclerotic disorders, wound healing and tumor angiogenesis. Gain-of-function mutation in this gene is linked with Penttinen syndrome, which is an autosomal-dominant disorder. Release of PGDFB by endothelial cells results in the activation of this receptor, which is essential for the recruitment of PDGFRB+ vascular smooth muscle cells and pericytes during blood vessel formation. Loss-of-function mutation in this gene results in the neurodegenerative disorder primary familial brain calcification (PFBC).
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Features and Benefits
Evaluate our antibodies with complete peace of mind. If the antibody does not perform in your application, we will issue a full credit or replacement antibody. Learn more.
General description
PDGF Receptor ? encodes a cell surface tyrosine kinase receptor for members of the platelet-derived growth factor family. These growth factors are mitogens for cells of mesenchymal origin. The identity of the growth factor bound to a receptor monomer determines whether the functional receptor is a homodimer or a heterodimer, composed of both platelet-derived growth factor receptor alpha and beta polypeptides. This gene is flanked on chromosome 5 by the genes for granulocyte-macrophage colony-stimulating factor and macrophage-colony stimulating factor receptor; all three genes may be implicated in the 5-q syndrome. A translocation between chromosomes 5 and 12, that fuses this gene to that of the translocation, ETV6, leukemia gene, results in chronic myeloproliferative disorder with eosinophilia.
Platelet derived growth factor receptor β (PDGFRB) is encoded by the gene mapped to human chromosome 5q32. The encoded protein is a type III tyrosine kinase receptor, which exists as a homodimer. This receptor interacts with multiple ligands, but its main physiological ligand is PDGF-BB homodimer.
Immunogen
Peptide sequence around aa. 749-753 (V-D-Y-V-P), according to the protein PDGFRB.
Physical form
Solution in phosphate-buffered saline containing 0.02% sodium azide and 50% glycerol
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存储类别
10 - Combustible liquids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
新产品
此项目有
Mutations in PDGFRB cause autosomal-dominant infantile myofibromatosis.
Martignetti J A, et al.
American Journal of Human Genetics, 92(6), 1001-1007 (2013)
Michael Vanlandewijck et al.
PloS one, 10(11), e0143407-e0143407 (2015-11-26)
Primary Familial Brain Calcification (PFBC), a neurodegenerative disease characterized by progressive pericapillary calcifications, has recently been linked to heterozygous mutations in PDGFB and PDGFRB genes. Here, we functionally analyzed several of these mutations in vitro. All six analyzed PDGFB mutations
Jennifer J Johnston et al.
American journal of human genetics, 97(3), 465-474 (2015-08-19)
Penttinen syndrome is a distinctive disorder characterized by a prematurely aged appearance with lipoatrophy, epidermal and dermal atrophy along with hypertrophic lesions that resemble scars, thin hair, proptosis, underdeveloped cheekbones, and marked acro-osteolysis. All individuals have been simplex cases. Exome
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