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Rabbit Anti-TPOR/FITC Conjugated antibody
background:
In 1990 an oncogene, v-mpl, was identified from the murine myeloproliferative leukemia virus that was capable of immortalizing bone marrow hematopoietic cells from different lineages. In 1992 the human homologue, named, c-mpl, was cloned. Sequence data revealed that c-mpl encoded a protein that was homologous with members of the hematopoietic receptor superfamily. Presence of anti-sense oligodeoxynucleotides of c-mpl inhibited megakaryocyte colony formation. The ligand for c-mpl, thrombopoietin, was cloned in 1994. Thrombopoietin was shown to be the major regulator of megakaryocytopoiesis and platelet formation. The protein encoded by the c-mpl gene, CD110, is a 635 amino acid transmembrane domain, with two extracellular cytokine receptor domains and two intracellular cytokine receptor box motifs . TPO-R deficient mice were severely thrombocytopenic, emphasizing the important role of CD110 and thrombopoietin in megakaryocyte and platelet formation. Upon binding of thrombopoietin CD110 is dimerized and the JAK family of non-receptor tyrosine kinases, as well as the STAT family, the MAPK family, the adaptor protein Shc and the receptors themselves become tyrosine phosphorylated. [provided by RefSeq, Jul 2008]
Function:
Receptor for thrombopoietin. May represent a regulatory molecule specific for TPO-R-dependent immune responses.
Subunit:
Interacts with ATXN2L
Subcellular Location:
Membrane.
Tissue Specificity:
Expressed at a low level in a large number of cells of hematopoietic origin. Isoform 1 and isoform 2 are always found to be coexpressed.
Post-translational modifications:
Ubiquitination at Lys-553 and Lys-573 targets MPL for degradation by both the lysosomal and proteasomal pathways. The E3 ubiquitin-protein ligase CBL significantly contributes to this ubiquitination.
DISEASE:
Defects in MPL are a cause of congenital amegakaryocytic thrombocytopenia (CAMT) [MIM:604498]. CAMT is a disease characterized by isolated thrombocytopenia and megakaryocytopenia with no physical anomalies.
Similarity:
Belongs to the type I cytokine receptor family. Type 1 subfamily.
Contains 2 fibronectin type-III domains.
Database links:
Entrez Gene: 4352 Human
Entrez Gene: 1796 Mouse
Entrez Gene: 366455 Rat
Omim: 159530 Human
SwissProt: P40238 Human
SwissProt: Q08351 Mouse
Unigene: 82906 Human
Unigene: 4864 Mouse
Unigene: 198731 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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