LIFR_HUMAN » Leukemia inhibitory factor receptor

LIFR_HUMAN » Leukemia inhibitory factor receptor
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
LIFR_HUMAN » Leukemia inhibitory factor receptor » LIF receptor; LIF-R;
Hydrophobic Thickness 35.2 ± 4.4 Å
Tilt Angle 0 ± 6°
ΔGtransfer -25.1 kcal/mol
ΔGfold -21.1 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology Out
TM Segments 833-857 (833-862)
Pathways

Cytokine-cytokine receptor interaction (KEGG)

Jak-STAT signaling pathway (KEGG)

PDB 3e0g (52-534)
OPM none
Complexes none
Interactions

IL31R, Complex: LIFR:IL31R, PubMed

IL6RB, Complex: IL6RB:LIFR:LIF, PubMed

IL6RB, Complex: LIFR:IL6RB, PubMed

Domains

AA: 434-523, PDBID: 3E0G, Subunit A, Seq Identity:100%, Fibronectin type III domain

UniProt annotation for LIFR_HUMAN » Leukemia inhibitory factor receptor
FUNCTION: Signal-transducing molecule. May have a common pathway with IL6ST. The soluble form inhibits the biological activity of LIF by blocking its binding to receptors on target cells.

SUBUNIT: Heterodimer composed of LIFR and IL6ST. The heterodimer formed by LIFR and IL6ST interacts with the complex formed by CNTF and CNTFR.

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: Stueve-Wiedemann syndrome (STWS) OMIM: Severe autosomal recessive condition and belongs to the group of the bent-bone dysplasias. SWS is characterized by bowing of the lower limbs, with internal cortical thickening, wide metaphyses with abnormal trabecular pattern, and camptodactyly. Additional features include feeding and swallowing difficulties, as well as respiratory distress and hyperthermic episodes, which cause death in the first months of life. The rare survivors develop progressive scoliosis, spontaneous fractures, bowing of the lower limbs, with prominent joints and dysautonomia symptoms, including temperature instability, absent corneal and patellar reflexes, and smooth tongue. caused by mutations affecting the gene represented in this entry.

DISEASE: Note=A chromosomal aberration involving LIFR is found in salivary gland pleiomorphic adenomas, the most common benign epithelial tumors of the salivary gland. Translocation t(5;8)(p13;q12) with PLAG1.

UniProt features for LIFR_HUMAN » Leukemia inhibitory factor receptor
SIGNAL 1 44 Potential.
CHAIN 45 1097 Leukemia inhibitory factor receptor.
DOMAIN 46 131 Fibronectin type-III 1.
DOMAIN 332 428 Fibronectin type-III 2.
DOMAIN 433 530 Fibronectin type-III 3.
DOMAIN 535 625 Fibronectin type-III 4.
DOMAIN 627 719 Fibronectin type-III 5.
DOMAIN 724 829 Fibronectin type-III 6.
MOTIF 519 523 WSXWS motif.
MOTIF 869 877 Box 1 motif.
DISULFID 55 65
DISULFID 82 90
DISULFID 213 270
DISULFID 341 351
DISULFID 466 511
Amino Acid Sequence for LIFR_HUMAN » Leukemia inhibitory factor receptor
MMDIYVCLKR PSWMVDNKRM RTASNFQWLL STFILLYLMN QVNSQKKGAP HDLKCVTNNL QVWNCSWKAP SGTGRGTDYE VCIENRSRSC YQLEKTSIKI PALSHGDYEI TINSLHDFGS STSKFTLNEQ NVSLIPDTPE ILNLSADFST STLYLKWNDR GSVFPHRSNV IWEIKVLRKE SMELVKLVTH NTTLNGKDTL HHWSWASDMP LECAIHFVEI RCYIDNLHFS GLEEWSDWSP VKNISWIPDS QTKVFPQDKV ILVGSDITFC CVSQEKVLSA LIGHTNCPLI HLDGENVAIK IRNISVSASS GTNVVFTTED NIFGTVIFAG YPPDTPQQLN CETHDLKEII CSWNPGRVTA LVGPRATSYT LVESFSGKYV RLKRAEAPTN ESYQLLFQML PNQEIYNFTL NAHNPLGRSQ STILVNITEK VYPHTPTSFK VKDINSTAVK LSWHLPGNFA KINFLCEIEI KKSNSVQEQR NVTIKGVENS SYLVALDKLN PYTLYTFRIR CSTETFWKWS KWSNKKQHLT TEASPSKGPD TWREWSSDGK NLIIYWKPLP INEANGKILS YNVSCSSDEE TQSLSEIPDP QHKAEIRLDK NDYIISVVAK NSVGSSPPSK IASMEIPNDD LKIEQVVGMG KGILLTWHYD PNMTCDYVIK WCNSSRSEPC LMDWRKVPSN STETVIESDE FRPGIRYNFF LYGCRNQGYQ LLRSMIGYIE ELAPIVAPNF TVEDTSADSI LVKWEDIPVE ELRGFLRGYL FYFGKGERDT SKMRVLESGR SDIKVKNITD ISQKTLRIAD LQGKTSYHLV LRAYTDGGVG PEKSMYVVTK ENSVGLIIAI LIPVAVAVIV GVVTSILCYR KREWIKETFY PDIPNPENCK ALQFQKSVCE GSSALKTLEM NPCTPNNVEV LETRSAFPKI EDTEIISPVA ERPEDRSDAE PENHVVVSYC PPIIEEEIPN PAADEAGGTA QVIYIDVQSM YQPQAKPEEE QENDPVGGAG YKPQMHLPIN STVEDIAAEE DLDKTAGYRP QANVNTWNLV SPDSPRSIDS NSEIVSFGSP CSINSRQFLI PPKDEDSPKS NGGGWSFTNF FQNKPND