TPOR_HUMAN » Thrombopoietin receptor

TPOR_HUMAN » Thrombopoietin receptor
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
TPOR_HUMAN » Thrombopoietin receptor » TPO-R; Myeloproliferative leukemia protein;Proto-oncogene c-Mpl;
Hydrophobic Thickness 36.4 ± 3.8 Å
Tilt Angle 1 ± 0°
ΔGtransfer -35.8 kcal/mol
ΔGfold -16.5 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC, Reactome
Topology Out
TM Segments 491-515 (486-515)
Pathways

Cytokine-cytokine receptor interaction (KEGG)

Hemostasis (Reactome)

Jak-STAT signaling pathway (KEGG)

PDB none
OPM none
Complexes none
Interactions none
Domains

AA: 25-128, PDBID: 1CN4, Subunit B, Seq Identity:30%, Erythropoietin receptor, ligand binding

AA: 391-475, PDBID: 1UEN, Subunit A, Seq Identity:24%, Fibronectin type III domain

UniProt annotation for TPOR_HUMAN » Thrombopoietin receptor
FUNCTION: Receptor for thrombopoietin. May represent a regulatory molecule specific for TPO-R-dependent immune responses.

SUBUNIT: Homodimer (PubMed). Interacts with ATXN2L.

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.

DOMAIN: The WSXWS motif appears to be necessary for proper protein folding and thereby efficient intracellular transport and cell- surface receptor binding.

DOMAIN: The box 1 motif is required for JAK interaction and/or activation.

DISEASE: Congenital amegakaryocytic thrombocytopenia (CAMT) OMIM: Disease characterized by isolated thrombocytopenia and megakaryocytopenia with no physical anomalies. mutations affecting the gene represented in this entry.

DISEASE: Thrombocythemia 2 (THCYT2) OMIM: A myeloproliferative disorder characterized by excessive platelet production, resulting in increased numbers of circulating platelets. It can be associated with spontaneous hemorrhages and thrombotic episodes. Note=The disease is caused by mutations affecting the gene represented in this entry.

DISEASE: Myelofibrosis with myeloid metaplasia (MMM) OMIM: A chronic myeloproliferative disorder characterized by replacement of the bone marrow by fibrous tissue, extramedullary hematopoiesis, anemia, leukoerythroblastosis and hepatosplenomegaly. mutations affecting the gene represented in this entry.

UniProt features for TPOR_HUMAN » Thrombopoietin receptor
SIGNAL 1 25 Potential.
CHAIN 26 635 Thrombopoietin receptor.
DOMAIN 133 215 Fibronectin type-III 1.
DOMAIN 394 481 Fibronectin type-III 2.
MOTIF 474 478 WSXWS motif.
MOTIF 528 536 Box 1 motif.
DISULFID 40 50 By similarity.
DISULFID 77 93 By similarity.
DISULFID 291 301 By similarity.
DISULFID 323 334 By similarity.
CROSSLNK 553 553 Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin).
CROSSLNK 573 573 Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin).
Amino Acid Sequence for TPOR_HUMAN » Thrombopoietin receptor
MPSWALFMVT SCLLLAPQNL AQVSSQDVSL LASDSEPLKC FSRTFEDLTC FWDEEEAAPS GTYQLLYAYP REKPRACPLS SQSMPHFGTR YVCQFPDQEE VRLFFPLHLW VKNVFLNQTR TQRVLFVDSV GLPAPPSIIK AMGGSQPGEL QISWEEPAPE ISDFLRYELR YGPRDPKNST GPTVIQLIAT ETCCPALQRP HSASALDQSP CAQPTMPWQD GPKQTSPSRE ASALTAEGGS CLISGLQPGN SYWLQLRSEP DGISLGGSWG SWSLPVTVDL PGDAVALGLQ CFTLDLKNVT CQWQQQDHAS SQGFFYHSRA RCCPRDRYPI WENCEEEEKT NPGLQTPQFS RCHFKSRNDS IIHILVEVTT APGTVHSYLG SPFWIHQAVR LPTPNLHWRE ISSGHLELEW QHPSSWAAQE TCYQLRYTGE GHQDWKVLEP PLGARGGTLE LRPRSRYRLQ LRARLNGPTY QGPWSSWSDP TRVETATETA WISLVTALHL VLGLSAVLGL LLLRWQFPAH YRRLRHALWP SLPDLHRVLG QYLRDTAALS PPKATVSDTC EEVEPSLLEI LPKSSERTPL PLCSSQAQMD YRRLQPSCLG TMPLSVCPPM AESGSCCTTH IANHSYLPLS YWQQP