描述：
PDGF Rα (plateletderived growth factor receptor alpha) is a type I transmembrane
glycoprotein in the class III subfamily of receptor tyrosine kinases (RTK) .
PDGF Rα and PDGF Rβ can form homoor heterodimeric receptors when engaged
by dimers of the PDGF family of growth factors, which include disulfidelinked
homodimers of PDGFA, B, C or D, or the heterodimer PDGFAB that is mainly
found in human platelets. While multiple in vitro ligandreceptor combinations have
been identified, in vivo evidence indicates that PDGF Rα primarily binds PDGFAA
and PDGFCC, while PDGF Rβ primarily binds PDGFBB and probably PDGFDD.
Like all class III RTKs, the extracellular domain (ECD) of mouse PDGF Rα (aa 25525)
contains five immunoglobulinlike domains, while the intracellular region
contains a split tyrosine kinase domain (aa 593 -954). Within the ECD, mouse
PDGF Rα shares 85%, 93%, 84%, 84%, and 81% aa sequence identity with human,
rat, equine, canine and bovine PDGF Rα respectively. PDGF Rα autophosphorylates
upon dimerization, activating signaling cascades in PI 3kinase RasMAP kinase,
and PLCγ pathways. Signaling is down-regulated by SHP2 phosphatase activity
and by receptor endocytosis and lysosomal degradation. PDGF Rα is
expressed at low levels in most mesenchymal cells, but is strongly expressed in
oligodendrocyte, lung, skin and intestinal progenitor cells and induced by
inflammation or growth in culture. During development, mesenchymal cells
expressing PDGF Rα respond to local gradients of epithelially produced PDGFAA
or PDGFCC during formation of the cranial and cardiac neural crest, retina, gonads,
lung alveoli, intestinal villi, skin, hair follicles, skeleton, teeth, palate, and interstitial
kidney mesenchyme. Deletion of PDGF Rα in mice severely impairs mesenchymal
derivatives in both embryo and extraembryonic tissues, and high or low
PDGF Rα signaling in humans may result in spina bifida or cleft palate-type
malformations. Postnatally, PDGF Rα is implicated in gliomas and fibrotic disorders
of lung, heart and skin (scleroderma).
原厂资料：
PDGF Rα (plateletderived growth factor receptor alpha) is a type I transmembrane
glycoprotein in the class III subfamily of receptor tyrosine kinases (RTK) .
PDGF Rα and PDGF Rβ can form homoor heterodimeric receptors when engaged
by dimers of the PDGF family of growth factors, which include disulfidelinked
homodimers of PDGFA, B, C or D, or the heterodimer PDGFAB that is mainly
found in human platelets. While multiple in vitro ligandreceptor combinations have
been identified, in vivo evidence indicates that PDGF Rα primarily binds PDGFAA
and PDGFCC, while PDGF Rβ primarily binds PDGFBB and probably PDGFDD.
Like all class III RTKs, the extracellular domain (ECD) of mouse PDGF Rα (aa 25525)
contains five immunoglobulinlike domains, while the intracellular region
contains a split tyrosine kinase domain (aa 593 -954). Within the ECD, mouse
PDGF Rα shares 85%, 93%, 84%, 84%, and 81% aa sequence identity with human,
rat, equine, canine and bovine PDGF Rα respectively. PDGF Rα autophosphorylates
upon dimerization, activating signaling cascades in PI 3kinase RasMAP kinase,
and PLCγ pathways. Signaling is down-regulated by SHP2 phosphatase activity
and by receptor endocytosis and lysosomal degradation. PDGF Rα is
expressed at low levels in most mesenchymal cells, but is strongly expressed in
oligodendrocyte, lung, skin and intestinal progenitor cells and induced by
inflammation or growth in culture. During development, mesenchymal cells
expressing PDGF Rα respond to local gradients of epithelially produced PDGFAA
or PDGFCC during formation of the cranial and cardiac neural crest, retina, gonads,
lung alveoli, intestinal villi, skin, hair follicles, skeleton, teeth, palate, and interstitial
kidney mesenchyme. Deletion of PDGF Rα in mice severely impairs mesenchymal
derivatives in both embryo and extraembryonic tissues, and high or low
PDGF Rα signaling in humans may result in spina bifida or cleft palate-type
malformations. Postnatally, PDGF Rα is implicated in gliomas and fibrotic disorders
of lung, heart and skin (scleroderma).
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