anti-CD117 (human), Rabbit Monoclonal (RM359)

CD117 (c-Kit;; Stem cell growth factor receptor; SCFR) is a trans membrane type III tyrosine kinase receptor encoded by the c-Kit proto-oncogene, located on chromosome 4 in humans. It is expressed in hematopoietic stem cells, germ cells, mast cells and gastrointestinal tract cajal cells. Overexpression or mutations of this protein can lead to cancer. Upon binding of its ligand stem cell factor (SCF), CD117 dimerizes, resulting in receptor activation and autophosphorylation of various tyrosine residues including tyrosine 703 located on the cytoplasmic domain of the receptor. This modification allows docking of Grb2 and activation of the Ras/ERK signaling pathway. SCF/CD117 can activate multiple downstream signaling pathways including PI3K, PLC-gamma and JAK/STAT. Signaling through CD117 plays a role in cell survival, proliferation, and differentiation. CD117 receptor activation is essential for hematopoiesis, stem cell maintenance and gametogenesis. Activating mutations in this gene are associated with gastrointestinal stromal tumors, testicular seminoma, mast cell disease, melanoma, acute myeloid leukemia, while inactivating mutations are associated with the genetic defect piebaldism. Antibodies to CD117 are widely used in immunohistochemistry to help distinguish particular types of tumour in histological tissue sections.