NOTC3_HUMAN » Neurogenic locus notch homolog protein 3

NOTC3_HUMAN » Neurogenic locus notch homolog protein 3
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
NOTC3_HUMAN » Neurogenic locus notch homolog protein 3 » Notch 3;
Hydrophobic Thickness 33.8 ± 1.2 Å
Tilt Angle 28 ± 15°
ΔGtransfer -27.3 kcal/mol
ΔGfold -22.0 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology Out
TM Segments 1643-1665 (1643-1670)
Pathways

A third proteolytic cleavage releases NICD (Reactome)

Dorso-ventral axis formation (KEGG)

Gene Expression (Reactome)

NICD traffics to nucleus (Reactome)

Notch signaling pathway (KEGG)

Receptor-ligand binding initiates the second proteolytic cleavage of Notch receptor (Reactome)

Signal Transduction (Reactome)

PDB none
OPM none
Complexes none
Interactions

DLL1, Complex: NOTC3:DLL1, PubMed

GGT6, Complex: GGT6:NOTC3

JAG1, Complex: NOTC3:JAG1, PubMed

JAG2, Complex: NOTC3:JAG2, PubMed

LRP1, Complex: LRP1:NOTC3, PubMed

NOTC1, Complex: NOTC1:NOTC3, PubMed

PTCRA, Complex: PTCRA:NOTC3, PubMed

RET, Complex: RET:NOTC3

SPTC2, Complex: SPTC2:NOTC3

TM108, Complex: TM108:NOTC3

Domains

AA: 43-75, PDBID: 4XBM, Subunit B, Seq Identity:50%, EGF-like domain

AA: 123-154, PDBID: 4XBM, Subunit B, Seq Identity:52%, EGF-like domain

AA: 158-201, PDBID: 4XL1, Subunit A, Seq Identity:58%, Calcium-binding EGF domain

AA: 201-232, PDBID: 1BF9, Subunit A, Seq Identity:58%, EGF-like domain

AA: 240-270, PDBID: 4XBM, Subunit B, Seq Identity:50%, EGF-like domain

AA: 274-315, PDBID: 1TOZ, Subunit A, Seq Identity:52%, Calcium-binding EGF domain

AA: 317-348, PDBID: 1TOZ, Subunit A, Seq Identity:50%, EGF-like domain

AA: 355-385, PDBID: 1TOZ, Subunit A, Seq Identity:48%, EGF-like domain

AA: 391-430, PDBID: 1TOZ, Subunit A, Seq Identity:81%, Calcium-binding EGF domain

AA: 431-467, PDBID: 2RQZ, Subunit A, Seq Identity:68%, Calcium-binding EGF domain

AA: 473-503, PDBID: 4XBM, Subunit B, Seq Identity:60%, EGF-like domain

AA: 511-541, PDBID: 4XBM, Subunit B, Seq Identity:60%, EGF-like domain

AA: 549-578, PDBID: 4XBM, Subunit B, Seq Identity:48%, EGF-like domain

AA: 586-616, PDBID: 4BDW, Subunit A, Seq Identity:55%, EGF-like domain

AA: 666-686, PDBID: 2YGQ, Subunit A, Seq Identity:52%, Human growth factor-like EGF

AA: 704-724, PDBID: 2YGQ, Subunit A, Seq Identity:39%, Human growth factor-like EGF

AA: 775-806, PDBID: 4BDW, Subunit A, Seq Identity:44%, EGF-like domain

AA: 810-848, PDBID: 4XL1, Subunit A, Seq Identity:57%, Calcium-binding EGF domain

AA: 853-883, PDBID: 4XBM, Subunit B, Seq Identity:63%, EGF-like domain

AA: 896-916, PDBID: 4D90, Subunit A, Seq Identity:47%, Human growth factor-like EGF

AA: 926-958, PDBID: 4XBM, Subunit B, Seq Identity:54%, EGF-like domain

AA: 1004-1032, PDBID: 4XBM, Subunit B, Seq Identity:50%, EGF-like domain

AA: 1051-1080, PDBID: 1AUT, Subunit L, Seq Identity:35%, EGF-like domain

AA: 1088-1118, PDBID: 3H5C, Subunit B, Seq Identity:51%, EGF-like domain

AA: 1126-1155, PDBID: 4XL1, Subunit D, Seq Identity:51%, EGF-like domain

AA: 1164-1201, PDBID: 3H5C, Subunit B, Seq Identity:45%, EGF-like domain

AA: 1205-1255, PDBID: 2RQZ, Subunit A, Seq Identity:36%, Calcium-binding EGF domain

AA: 1339-1371, PDBID: 1TOZ, Subunit A, Seq Identity:41%, EGF-like domain

AA: 1384-1418, PDBID: 4ZLP, Subunit A, Seq Identity:100%, LNR domain

AA: 1425-1459, PDBID: 4ZLP, Subunit A, Seq Identity:100%, LNR domain

AA: 1464-1501, PDBID: 4ZLP, Subunit A, Seq Identity:100%, LNR domain

AA: 1505-1560, PDBID: 4ZLP, Subunit A, Seq Identity:100%, NOTCH protein

AA: 1577-1637, PDBID: 4ZLP, Subunit A, Seq Identity:100%, NOTCH protein

AA: 1812-1903, PDBID: 1OT8, Subunit C, Seq Identity:65%, Ankyrin repeats (3 copies)

AA: 1883-1969, PDBID: 1YYH, Subunit B, Seq Identity:88%, Ankyrin repeats (3 copies)

AA: 1910-2003, PDBID: 1YYH, Subunit B, Seq Identity:85%, Ankyrin repeats (3 copies)

AA: 2211-2274, Domain of unknown function (DUF3454)

UniProt annotation for NOTC3_HUMAN » Neurogenic locus notch homolog protein 3
FUNCTION: Functions as a receptor for membrane-bound ligands Jagged1, Jagged2 and Delta1 to regulate cell-fate determination. Upon ligand activation through the released notch intracellular domain (NICD) it forms a transcriptional activator complex with RBPJ/RBPSUH and activates genes of the enhancer of split locus. Affects the implementation of differentiation, proliferation and apoptotic programs (By similarity).

SUBUNIT: Heterodimer of a C-terminal fragment N(TM) and a N- terminal fragment N(EC) which are probably linked by disulfide bonds (By similarity). Interacts with MAML1, MAML2 and MAML3 which act as transcriptional coactivators for NOTCH3. Interacts with PSMA1. Interacts with HIF1AN.

TISSUE SPECIFICITY: Ubiquitously expressed in fetal and adult tissues.

DISEASE: Cerebral arteriopathy with subcortical infarcts and leukoencephalopathy, autosomal dominant (CADASIL) OMIM: A cerebrovascular disease characterized by multiple subcortical infarcts, pseudobulbar palsy, dementia, and the presence of granular deposits in small cerebral arteries producing ischemic stroke. mutations affecting the gene represented in this entry.

DISEASE: Myofibromatosis, infantile 2 (IMF2) OMIM: A rare mesenchymal disorder characterized by the development of benign tumors in the skin, striated muscles, bones, and, more rarely, visceral organs. Subcutaneous or soft tissue nodules commonly involve the skin of the head, neck, and trunk. Skeletal and muscular lesions occur in about half of the patients. Lesions may be solitary or multicentric, and they may be present at birth or become apparent in early infancy or occasionally in adult life. Visceral lesions are associated with high morbidity and mortality. mutations affecting the gene represented in this entry.

Amino Acid Sequence for NOTC3_HUMAN » Neurogenic locus notch homolog protein 3
MGPGARGRRR RRRPMSPPPP PPPVRALPLL LLLAGPGAAA PPCLDGSPCA NGGRCTQLPS REAACLCPPG WVGERCQLED PCHSGPCAGR GVCQSSVVAG TARFSCRCPR GFRGPDCSLP DPCLSSPCAH GARCSVGPDG RFLCSCPPGY QGRSCRSDVD ECRVGEPCRH GGTCLNTPGS FRCQCPAGYT GPLCENPAVP CAPSPCRNGG TCRQSGDLTY DCACLPGFEG QNCEVNVDDC PGHRCLNGGT CVDGVNTYNC QCPPEWTGQF CTEDVDECQL QPNACHNGGT CFNTLGGHSC VCVNGWTGES CSQNIDDCAT AVCFHGATCH DRVASFYCAC PMGKTGLLCH LDDACVSNPC HEDAICDTNP VNGRAICTCP PGFTGGACDQ DVDECSIGAN PCEHLGRCVN TQGSFLCQCG RGYTGPRCET DVNECLSGPC RNQATCLDRI GQFTCICMAG FTGTYCEVDI DECQSSPCVN GGVCKDRVNG FSCTCPSGFS GSTCQLDVDE CASTPCRNGA KCVDQPDGYE CRCAEGFEGT LCDRNVDDCS PDPCHHGRCV DGIASFSCAC APGYTGTRCE SQVDECRSQP CRHGGKCLDL VDKYLCRCPS GTTGVNCEVN IDDCASNPCT FGVCRDGINR YDCVCQPGFT GPLCNVEINE CASSPCGEGG SCVDGENGFR CLCPPGSLPP LCLPPSHPCA HEPCSHGICY DAPGGFRCVC EPGWSGPRCS QSLARDACES QPCRAGGTCS SDGMGFHCTC PPGVQGRQCE LLSPCTPNPC EHGGRCESAP GQLPVCSCPQ GWQGPRCQQD VDECAGPAPC GPHGICTNLA GSFSCTCHGG YTGPSCDQDI NDCDPNPCLN GGSCQDGVGS FSCSCLPGFA GPRCARDVDE CLSNPCGPGT CTDHVASFTC TCPPGYGGFH CEQDLPDCSP SSCFNGGTCV DGVNSFSCLC RPGYTGAHCQ HEADPCLSRP CLHGGVCSAA HPGFRCTCLE SFTGPQCQTL VDWCSRQPCQ NGGRCVQTGA YCLCPPGWSG RLCDIRSLPC REAAAQIGVR LEQLCQAGGQ CVDEDSSHYC VCPEGRTGSH CEQEVDPCLA QPCQHGGTCR GYMGGYMCEC LPGYNGDNCE DDVDECASQP CQHGGSCIDL VARYLCSCPP GTLGVLCEIN EDDCGPGPPL DSGPRCLHNG TCVDLVGGFR CTCPPGYTGL RCEADINECR SGACHAAHTR DCLQDPGGGF RCLCHAGFSG PRCQTVLSPC ESQPCQHGGQ CRPSPGPGGG LTFTCHCAQP FWGPRCERVA RSCRELQCPV GVPCQQTPRG PRCACPPGLS GPSCRSFPGS PPGASNASCA AAPCLHGGSC RPAPLAPFFR CACAQGWTGP RCEAPAAAPE VSEEPRCPRA ACQAKRGDQR CDRECNSPGC GWDGGDCSLS VGDPWRQCEA LQCWRLFNNS RCDPACSSPA CLYDNFDCHA GGRERTCNPV YEKYCADHFA DGRCDQGCNT EECGWDGLDC ASEVPALLAR GVLVLTVLLP PEELLRSSAD FLQRLSAILR TSLRFRLDAH GQAMVFPYHR PSPGSEPRAR RELAPEVIGS VVMLEIDNRL CLQSPENDHC FPDAQSAADY LGALSAVERL DFPYPLRDVR GEPLEPPEPS VPLLPLLVAG AVLLLVILVL GVMVARRKRE HSTLWFPEGF SLHKDVASGH KGRREPVGQD ALGMKNMAKG ESLMGEVATD WMDTECPEAK RLKVEEPGMG AEEAVDCRQW TQHHLVAADI RVAPAMALTP PQGDADADGM DVNVRGPDGF TPLMLASFCG GALEPMPTEE DEADDTSASI ISDLICQGAQ LGARTDRTGE TALHLAARYA RADAAKRLLD AGADTNAQDH SGRTPLHTAV TADAQGVFQI LIRNRSTDLD ARMADGSTAL ILAARLAVEG MVEELIASHA DVNAVDELGK SALHWAAAVN NVEATLALLK NGANKDMQDS KEETPLFLAA REGSYEAAKL LLDHFANREI TDHLDRLPRD VAQERLHQDI VRLLDQPSGP RSPPGPHGLG PLLCPPGAFL PGLKAAQSGS KKSRRPPGKA GLGPQGPRGR GKKLTLACPG PLADSSVTLS PVDSLDSPRP FGGPPASPGG FPLEGPYAAA TATAVSLAQL GGPGRAGLGR QPPGGCVLSL GLLNPVAVPL DWARLPPPAP PGPSFLLPLA PGPQLLNPGT PVSPQERPPP YLAVPGHGEE YPVAGAHSSP PKARFLRVPS EHPYLTPSPE SPEHWASPSP PSLSDWSEST PSPATATGAM ATTTGALPAQ PLPLSVPSSL AQAQTQLGPQ PEVTPKRQVL A