ID ITIH6_HUMAN Reviewed; 1313 AA.
AC Q6UXX5; A6NN03;
DT 01-MAY-2007, integrated into UniProtKB/Swiss-Prot.
DT 05-JUL-2004, sequence version 1.
DT 27-MAR-2024, entry version 142.
DE RecName: Full=Inter-alpha-trypsin inhibitor heavy chain H6;
DE AltName: Full=Inter-alpha-trypsin inhibitor heavy chain H5-like protein;
DE Short=Inter-alpha inhibitor H5-like protein;
DE Flags: Precursor;
GN Name=ITIH6; Synonyms=ITIH5L; ORFNames=UNQ6369/PRO21074;
OS Homo sapiens (Human).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae;
OC Homo.
OX NCBI_TaxID=9606;
RN [1]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RX PubMed=12975309; DOI=10.1101/gr.1293003;
RA Clark H.F., Gurney A.L., Abaya E., Baker K., Baldwin D.T., Brush J.,
RA Chen J., Chow B., Chui C., Crowley C., Currell B., Deuel B., Dowd P.,
RA Eaton D., Foster J.S., Grimaldi C., Gu Q., Hass P.E., Heldens S., Huang A.,
RA Kim H.S., Klimowski L., Jin Y., Johnson S., Lee J., Lewis L., Liao D.,
RA Mark M.R., Robbie E., Sanchez C., Schoenfeld J., Seshagiri S., Simmons L.,
RA Singh J., Smith V., Stinson J., Vagts A., Vandlen R.L., Watanabe C.,
RA Wieand D., Woods K., Xie M.-H., Yansura D.G., Yi S., Yu G., Yuan J.,
RA Zhang M., Zhang Z., Goddard A.D., Wood W.I., Godowski P.J., Gray A.M.;
RT "The secreted protein discovery initiative (SPDI), a large-scale effort to
RT identify novel human secreted and transmembrane proteins: a bioinformatics
RT assessment.";
RL Genome Res. 13:2265-2270(2003).
RN [2]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RX PubMed=15772651; DOI=10.1038/nature03440;
RA Ross M.T., Grafham D.V., Coffey A.J., Scherer S., McLay K., Muzny D.,
RA Platzer M., Howell G.R., Burrows C., Bird C.P., Frankish A., Lovell F.L.,
RA Howe K.L., Ashurst J.L., Fulton R.S., Sudbrak R., Wen G., Jones M.C.,
RA Hurles M.E., Andrews T.D., Scott C.E., Searle S., Ramser J., Whittaker A.,
RA Deadman R., Carter N.P., Hunt S.E., Chen R., Cree A., Gunaratne P.,
RA Havlak P., Hodgson A., Metzker M.L., Richards S., Scott G., Steffen D.,
RA Sodergren E., Wheeler D.A., Worley K.C., Ainscough R., Ambrose K.D.,
RA Ansari-Lari M.A., Aradhya S., Ashwell R.I., Babbage A.K., Bagguley C.L.,
RA Ballabio A., Banerjee R., Barker G.E., Barlow K.F., Barrett I.P.,
RA Bates K.N., Beare D.M., Beasley H., Beasley O., Beck A., Bethel G.,
RA Blechschmidt K., Brady N., Bray-Allen S., Bridgeman A.M., Brown A.J.,
RA Brown M.J., Bonnin D., Bruford E.A., Buhay C., Burch P., Burford D.,
RA Burgess J., Burrill W., Burton J., Bye J.M., Carder C., Carrel L.,
RA Chako J., Chapman J.C., Chavez D., Chen E., Chen G., Chen Y., Chen Z.,
RA Chinault C., Ciccodicola A., Clark S.Y., Clarke G., Clee C.M., Clegg S.,
RA Clerc-Blankenburg K., Clifford K., Cobley V., Cole C.G., Conquer J.S.,
RA Corby N., Connor R.E., David R., Davies J., Davis C., Davis J., Delgado O.,
RA Deshazo D., Dhami P., Ding Y., Dinh H., Dodsworth S., Draper H.,
RA Dugan-Rocha S., Dunham A., Dunn M., Durbin K.J., Dutta I., Eades T.,
RA Ellwood M., Emery-Cohen A., Errington H., Evans K.L., Faulkner L.,
RA Francis F., Frankland J., Fraser A.E., Galgoczy P., Gilbert J., Gill R.,
RA Gloeckner G., Gregory S.G., Gribble S., Griffiths C., Grocock R., Gu Y.,
RA Gwilliam R., Hamilton C., Hart E.A., Hawes A., Heath P.D., Heitmann K.,
RA Hennig S., Hernandez J., Hinzmann B., Ho S., Hoffs M., Howden P.J.,
RA Huckle E.J., Hume J., Hunt P.J., Hunt A.R., Isherwood J., Jacob L.,
RA Johnson D., Jones S., de Jong P.J., Joseph S.S., Keenan S., Kelly S.,
RA Kershaw J.K., Khan Z., Kioschis P., Klages S., Knights A.J., Kosiura A.,
RA Kovar-Smith C., Laird G.K., Langford C., Lawlor S., Leversha M., Lewis L.,
RA Liu W., Lloyd C., Lloyd D.M., Loulseged H., Loveland J.E., Lovell J.D.,
RA Lozado R., Lu J., Lyne R., Ma J., Maheshwari M., Matthews L.H.,
RA McDowall J., McLaren S., McMurray A., Meidl P., Meitinger T., Milne S.,
RA Miner G., Mistry S.L., Morgan M., Morris S., Mueller I., Mullikin J.C.,
RA Nguyen N., Nordsiek G., Nyakatura G., O'dell C.N., Okwuonu G., Palmer S.,
RA Pandian R., Parker D., Parrish J., Pasternak S., Patel D., Pearce A.V.,
RA Pearson D.M., Pelan S.E., Perez L., Porter K.M., Ramsey Y., Reichwald K.,
RA Rhodes S., Ridler K.A., Schlessinger D., Schueler M.G., Sehra H.K.,
RA Shaw-Smith C., Shen H., Sheridan E.M., Shownkeen R., Skuce C.D.,
RA Smith M.L., Sotheran E.C., Steingruber H.E., Steward C.A., Storey R.,
RA Swann R.M., Swarbreck D., Tabor P.E., Taudien S., Taylor T., Teague B.,
RA Thomas K., Thorpe A., Timms K., Tracey A., Trevanion S., Tromans A.C.,
RA d'Urso M., Verduzco D., Villasana D., Waldron L., Wall M., Wang Q.,
RA Warren J., Warry G.L., Wei X., West A., Whitehead S.L., Whiteley M.N.,
RA Wilkinson J.E., Willey D.L., Williams G., Williams L., Williamson A.,
RA Williamson H., Wilming L., Woodmansey R.L., Wray P.W., Yen J., Zhang J.,
RA Zhou J., Zoghbi H., Zorilla S., Buck D., Reinhardt R., Poustka A.,
RA Rosenthal A., Lehrach H., Meindl A., Minx P.J., Hillier L.W., Willard H.F.,
RA Wilson R.K., Waterston R.H., Rice C.M., Vaudin M., Coulson A., Nelson D.L.,
RA Weinstock G., Sulston J.E., Durbin R.M., Hubbard T., Gibbs R.A., Beck S.,
RA Rogers J., Bentley D.R.;
RT "The DNA sequence of the human X chromosome.";
RL Nature 434:325-337(2005).
RN [3]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RA Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M.,
RA Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J.,
RA Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S.,
RA Turner R., Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H.,
RA Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K.,
RA Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D.,
RA Hunkapiller M.W., Myers E.W., Venter J.C.;
RL Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases.
CC -!- INTERACTION:
CC Q6UXX5; Q49AR9: ANKS1A; NbExp=3; IntAct=EBI-11028396, EBI-11954519;
CC Q6UXX5; A0A024RCZ4: C6orf1; NbExp=3; IntAct=EBI-11028396, EBI-23226736;
CC Q6UXX5; Q03828: EVX2; NbExp=3; IntAct=EBI-11028396, EBI-17280301;
CC Q6UXX5; Q5T749: KPRP; NbExp=3; IntAct=EBI-11028396, EBI-10981970;
CC Q6UXX5; Q3LI64: KRTAP6-1; NbExp=3; IntAct=EBI-11028396, EBI-12111050;
CC Q6UXX5; O43711: TLX3; NbExp=3; IntAct=EBI-11028396, EBI-3939165;
CC Q6UXX5; O95231: VENTX; NbExp=3; IntAct=EBI-11028396, EBI-10191303;
CC Q6UXX5; Q96IQ9: ZNF414; NbExp=3; IntAct=EBI-11028396, EBI-744257;
CC -!- SUBCELLULAR LOCATION: Secreted {ECO:0000250}.
CC -!- SIMILARITY: Belongs to the ITIH family. {ECO:0000305}.
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DR EMBL; AY358170; AAQ88537.1; -; mRNA.
DR EMBL; Z98046; -; NOT_ANNOTATED_CDS; Genomic_DNA.
DR EMBL; AL022575; -; NOT_ANNOTATED_CDS; Genomic_DNA.
DR EMBL; CH471154; EAW93186.1; -; Genomic_DNA.
DR CCDS; CCDS14361.1; -.
DR RefSeq; NP_940912.1; NM_198510.2.
DR AlphaFoldDB; Q6UXX5; -.
DR SMR; Q6UXX5; -.
DR BioGRID; 131428; 12.
DR IntAct; Q6UXX5; 9.
DR STRING; 9606.ENSP00000218436; -.
DR GlyCosmos; Q6UXX5; 6 sites, No reported glycans.
DR GlyGen; Q6UXX5; 7 sites, 1 O-linked glycan (1 site).
DR iPTMnet; Q6UXX5; -.
DR PhosphoSitePlus; Q6UXX5; -.
DR BioMuta; ITIH6; -.
DR DMDM; 74762375; -.
DR EPD; Q6UXX5; -.
DR MassIVE; Q6UXX5; -.
DR PaxDb; 9606-ENSP00000218436; -.
DR PeptideAtlas; Q6UXX5; -.
DR ProteomicsDB; 67671; -.
DR Antibodypedia; 62222; 13 antibodies from 9 providers.
DR DNASU; 347365; -.
DR Ensembl; ENST00000218436.7; ENSP00000218436.6; ENSG00000102313.9.
DR GeneID; 347365; -.
DR KEGG; hsa:347365; -.
DR MANE-Select; ENST00000218436.7; ENSP00000218436.6; NM_198510.3; NP_940912.1.
DR UCSC; uc004dtj.2; human.
DR AGR; HGNC:28907; -.
DR CTD; 347365; -.
DR DisGeNET; 347365; -.
DR GeneCards; ITIH6; -.
DR HGNC; HGNC:28907; ITIH6.
DR HPA; ENSG00000102313; Tissue enhanced (skeletal).
DR neXtProt; NX_Q6UXX5; -.
DR OpenTargets; ENSG00000102313; -.
DR PharmGKB; PA134930661; -.
DR VEuPathDB; HostDB:ENSG00000102313; -.
DR eggNOG; ENOG502QPS2; Eukaryota.
DR GeneTree; ENSGT00940000162980; -.
DR HOGENOM; CLU_008101_1_0_1; -.
DR InParanoid; Q6UXX5; -.
DR OMA; LPRWASC; -.
DR OrthoDB; 608326at2759; -.
DR PhylomeDB; Q6UXX5; -.
DR TreeFam; TF328982; -.
DR PathwayCommons; Q6UXX5; -.
DR SignaLink; Q6UXX5; -.
DR BioGRID-ORCS; 347365; 4 hits in 771 CRISPR screens.
DR GenomeRNAi; 347365; -.
DR Pharos; Q6UXX5; Tdark.
DR PRO; PR:Q6UXX5; -.
DR Proteomes; UP000005640; Chromosome X.
DR RNAct; Q6UXX5; Protein.
DR Bgee; ENSG00000102313; Expressed in cartilage tissue and 13 other cell types or tissues.
DR Genevisible; Q6UXX5; HS.
DR GO; GO:0005576; C:extracellular region; IEA:UniProtKB-SubCell.
DR GO; GO:0004867; F:serine-type endopeptidase inhibitor activity; IEA:UniProtKB-KW.
DR GO; GO:0030212; P:hyaluronan metabolic process; IEA:InterPro.
DR CDD; cd01461; vWA_interalpha_trypsin_inhibitor; 1.
DR Gene3D; 3.40.50.410; von Willebrand factor, type A domain; 1.
DR InterPro; IPR010600; ITI_HC_C.
DR InterPro; IPR013694; VIT.
DR InterPro; IPR002035; VWF_A.
DR InterPro; IPR036465; vWFA_dom_sf.
DR PANTHER; PTHR10338; INTER-ALPHA-TRYPSIN INHIBITOR HEAVY CHAIN FAMILY MEMBER; 1.
DR PANTHER; PTHR10338:SF155; INTER-ALPHA-TRYPSIN INHIBITOR HEAVY CHAIN H6; 1.
DR Pfam; PF06668; ITI_HC_C; 1.
DR Pfam; PF08487; VIT; 1.
DR Pfam; PF00092; VWA; 1.
DR SMART; SM00609; VIT; 1.
DR SMART; SM00327; VWA; 1.
DR SUPFAM; SSF53300; vWA-like; 1.
DR PROSITE; PS51468; VIT; 1.
DR PROSITE; PS50234; VWFA; 1.
PE 1: Evidence at protein level;
KW Glycoprotein; Protease inhibitor; Reference proteome; Secreted;
KW Serine protease inhibitor; Signal.
FT SIGNAL 1..23
FT /evidence="ECO:0000255"
FT CHAIN 24..1313
FT /note="Inter-alpha-trypsin inhibitor heavy chain H6"
FT /id="PRO_0000285680"
FT DOMAIN 24..150
FT /note="VIT"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00801"
FT DOMAIN 283..469
FT /note="VWFA"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00219"
FT REGION 612..644
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT REGION 783..817
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT REGION 856..928
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT REGION 959..983
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 793..814
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 860..877
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 878..900
FT /note="Pro residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 901..925
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT CARBOHYD 83
FT /note="N-linked (GlcNAc...) asparagine"
FT /evidence="ECO:0000255"
FT CARBOHYD 374
FT /note="N-linked (GlcNAc...) asparagine"
FT /evidence="ECO:0000255"
FT CARBOHYD 540
FT /note="N-linked (GlcNAc...) asparagine"
FT /evidence="ECO:0000255"
FT CARBOHYD 594
FT /note="N-linked (GlcNAc...) asparagine"
FT /evidence="ECO:0000255"
FT CARBOHYD 971
FT /note="N-linked (GlcNAc...) asparagine"
FT /evidence="ECO:0000255"
FT CARBOHYD 1231
FT /note="N-linked (GlcNAc...) asparagine"
FT /evidence="ECO:0000255"
FT VARIANT 387
FT /note="G -> R (in dbSNP:rs34188213)"
FT /id="VAR_032042"
FT VARIANT 561
FT /note="R -> C (in dbSNP:rs34004499)"
FT /id="VAR_032043"
FT VARIANT 1041
FT /note="W -> S (in dbSNP:rs17316491)"
FT /id="VAR_032044"
FT VARIANT 1170
FT /note="G -> A (in dbSNP:rs35355718)"
FT /id="VAR_032045"
SQ SEQUENCE 1313 AA; 143187 MW; 4C63CF26AF187734 CRC64;
MSGWRYLICV SFLLTILLEL TYQGPPVPAS SSTKLLMTSY SMRSTVVSRY AHTLVTSVLF
NPHAEAHEAI FDLDLPHLAF ISNFTMTINN KVYIAEVKEK HQAKKIYEEA HQQGKTAAHV
GIRDRESEKF RISTSLAAGT EVTFSLAYEE LLQRHQGQYQ LVVSLRPGQL VKRLSIEVTV
SERTGISYVH IPPLRTGRLR TNAHASEVDS PPSTRIERGE TCVRITYCPT LQDQSSISGS
GIMADFLVQY DVVMEDIIGD VQIYDDYFIH YFAPRGLPPM EKNVVFVIDV SSSMFGTKME
QTKTAMNVIL SDLQANDYFN IISFSDTVNV WKAGGSIQAT IQNVHSAKDY LHCMEADGWT
DVNSALLAAA SVLNHSNQEP GRGPSVGRIP LIIFLTDGEP TAGVTTPSVI LSNVRQALGH
RVSLFSLAFG DDADFTLLRR LSLENRGIAR RIYEDTDAAL QLKGLYEEIS MPLLADVRLN
YLGGLVGASP WAVFPNYFGG SELVVAGQVQ PGKQELGIHL AARGPKDQLL VAHHSEGATN
NSQKAFGCPG EPAPNVAHFI RRLWAYVTIG ELLDAHFQAR DTTTRHLLAA KVLNLSLEYN
FVTPLTSLVM VQPKQASEET RRQTSTSAGP DTIMPSSSSR HGLGVSTAQP ALVPKVISPK
SRPVKPKFYL SSTTTASTKK MLSSKELEPL GESPHTLSMP TYPKAKIPAQ QDSGTLAQPT
LRTKPTILVP SNSGTLLPLK PGSLSHQNPD ILPTNSRTQV PPVKPGIPAS PKADTVKCVT
PLHSKPGAPS HPQLGALTSQ APKGLPQSRP GVSTLQVPKY PLHTRPRVPA PKTRNNMPHL
GPGILLSKTP KILLSLKPSA PPHQISTSIS LSKPETPNPH MPQTPLPPRP DRPRPPLPES
LSTFPNTISS STGPSSTTTT SVLGEPLPMP FTPTLPPGRF WHQYDLLPGP QRTRQVLGPS
RPGVPTMSLL NSSRPTPEGS PPNLPILLPS SILPEAISLL LLPEELELLS ESMVESKFVE
SLNPPAFYTF LTPDEDGSPN WDGNSEEILG GAGGSMESQG SSVGLAKGTL PSIFTFSSSV
DGDPHFVIQI PHSEEKICFT LNGHPGDLLQ LIEDPKAGLH VSGKLLGAPP RPGHKDQTRT
YFQIITVTTD KPRAYTITIS RSSISLRGEG TLRLSWDQPA LLKRPQLELY VAAAARLTLR
LGPYLEFLVL RHRYRHPSTL QLPHLGFYVA NGSGLSPSAR GLIGQFQHAD IRLVTGPMGP
CLRRHHGPDV PVILGKRLLK DSPRLLPRWA SCWLVKRSHV ELLLGHPYLS YVL
//
