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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 [4]
Laboratory:
- MPL gene mutation
Note: uncertain whether Met-1 or Met-8 is the initiator
Related
MPL gene mutation
thrombopoietin receptor or myeloproliferative leukemia (MPL) gene
thrombopoietin; megakaryocyte colony stimulating factor; C-MPL ligand; megakaryocyte growth & development factor (MGDF, THPO)
General
cluster-of-differentiation antigen; cluster designation antigen; CD antigen
cytokine receptor
glycoprotein
hematopoietin receptor superfamily
Properties
SIZE: entity length = 635 aa
MW = 71 kD
COMPARTMENT: cellular membrane
MOTIF: signal sequence {1-25}
cysteine residue {C40}
MODIFICATION: cysteine residue {C50}
cysteine residue {C50}
MODIFICATION: cysteine residue {C40}
cysteine residue {C77}
MODIFICATION: cysteine residue {C93}
cysteine residue {C93}
MODIFICATION: cysteine residue {C77}
N-glycosylation site {N117}
fibronectin type III domain or F3 module {133-215}
MOTIF: N-glycosylation site {N178}
cysteine residue {C291}
MODIFICATION: cysteine residue {C301}
N-glycosylation site {N298}
cysteine residue {C301}
MODIFICATION: cysteine residue {C291}
cysteine residue {C323}
MODIFICATION: cysteine residue {C334}
cysteine residue {C334}
MODIFICATION: cysteine residue {C323}
glutamate-rich region {335-338}
MOTIF: glutamate residue (SEVERAL)
N-glycosylation site {N358}
fibronectin type III domain or F3 module {394-481}
MOTIF: WSXWS motif {474-478}
transmembrane domain {492-513}
box1 motif
SITE: 528-536
FOR-BINDING-OF: Janus kinase (JAK)
Database Correlations
OMIM correlations
MORBIDMAP 159530
UniProt P40238
PFAM correlations
Entrez Gene 4352
Kegg hsa:4352
References
- UniProt :accession P40238
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=MPL
- http://www.pathologyoutlines.com/cdmarkers.html
21 June 2005
- Brooks M
FDA OKs Platelet Booster for Liver Disease Patients .
Medscape - May 21, 2018
https://www.medscape.com/viewarticle/897029