thrombopoietin receptor; TPO-R; myeloproliferative leukemia protein; C-mpl; CD110 (MPL, TPOR)

Function

• receptor for thrombopoietin
• induces megakaryocyte proliferation & differentiation
• prevents apoptosis
• may represent a regulatory molecule specific for TPO-R-dependent immune responses
• interacts with ATXN2L

Structure

• WSXWS motif appears to be necessary for proper protein folding & thus efficient intracellular transport & cell-surface receptor binding
• box 1 motif is required for JAK interaction &/or activation
• belongs to the type I cytokine receptor family, type 1 subfamily
• contains 2 fibronectin F3 modules

Compartment

membrane

Alternative splicing

named isoforms=2

Expression

• megakaryocytes & progenitors
• platelets
• expressed at a low level in a large number of cells of hematopoietic origin
• isoform 1 & isoform 2 are always coexpressed

Pathology

• defects in MPL are a cause of
  • congenital amegakaryocytic thrombocytopenia
  • essential thrombocythemia
  • myelofibrosis

Pharmacology

• avatrombopag (Doptelet) is a thrombopoietin receptor agonist that stimulates platelet production

Laboratory

• MPL gene mutation

Notes

uncertain whether Met-1 or Met-8 is the initiator

More general terms

• CD antigen
• hematopoietin receptor superfamily
• glycoprotein
• cytokine receptor

Additional terms

• thrombopoietin; megakaryocyte colony stimulating factor; C-MPL ligand; megakaryocyte growth & development factor (MGDF, THPO)

Database

• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:4352
• OMIM: https://mirror.omim.org/entry/159530
• OMIM: https://mirror.omim.org/entry/604498
• UniProt: http://www.uniprot.org/uniprot/P40238.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=4352