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Inter-alpha-trypsin inhibitor heavy chain H4 (ITI heavy chain H4) (ITI-HC4) (Inter-alpha-inhibitor heavy chain 4) (Inter-alpha-trypsin inhibitor family heavy chain-related protein) (IHRP) (Plasma kallikrein sensitive glycoprotein 120) (Gp120) (PK-120) [Cleaved into: 70 kDa inter-alpha-trypsin inhibitor heavy chain H4; 35 kDa inter-alpha-trypsin inhibitor heavy chain H4]

 ITIH4_HUMAN             Reviewed;         930 AA.
Q14624; B7Z545; E9PGN5; Q15135; Q9P190; Q9UQ54;
15-JUL-1998, integrated into UniProtKB/Swiss-Prot.
05-MAY-2009, sequence version 4.
25-OCT-2017, entry version 159.
RecName: Full=Inter-alpha-trypsin inhibitor heavy chain H4;
Short=ITI heavy chain H4;
Short=ITI-HC4;
Short=Inter-alpha-inhibitor heavy chain 4;
AltName: Full=Inter-alpha-trypsin inhibitor family heavy chain-related protein;
Short=IHRP;
AltName: Full=Plasma kallikrein sensitive glycoprotein 120;
Short=Gp120;
Short=PK-120;
Contains:
RecName: Full=70 kDa inter-alpha-trypsin inhibitor heavy chain H4;
Contains:
RecName: Full=35 kDa inter-alpha-trypsin inhibitor heavy chain H4;
Flags: Precursor;
Name=ITIH4; Synonyms=IHRP, ITIHL1, PK120; ORFNames=PRO1851;
Homo sapiens (Human).
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini;
Catarrhini; Hominidae; Homo.
NCBI_TaxID=9606;
[1]
NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), PARTIAL PROTEIN SEQUENCE, AND
TISSUE SPECIFICITY.
TISSUE=Liver;
PubMed=7805892; DOI=10.1016/0014-5793(94)01364-7;
Nishimura H., Kakizaki I., Muta T., Sasaki N., Pu P.X., Yamashita T.,
Nagasawa S.;
"cDNA and deduced amino acid sequence of human PK-120, a plasma
kallikrein-sensitive glycoprotein.";
FEBS Lett. 357:207-211(1995).
[2]
NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), PARTIAL PROTEIN SEQUENCE,
IDENTIFICATION, TISSUE SPECIFICITY, AND VARIANT ASN-85.
TISSUE=Liver;
PubMed=7775381;
Saguchi K., Tobe T., Hashimoto K., Sano Y., Nakano Y., Miura N.-H.,
Tomita M.;
"Cloning and characterization of cDNA for inter-alpha-trypsin
inhibitor family heavy chain-related protein (IHRP), a novel human
plasma glycoprotein.";
J. Biochem. 117:14-18(1995).
[3]
NUCLEOTIDE SEQUENCE [GENOMIC DNA] (ISOFORM 1).
PubMed=8797089; DOI=10.1093/oxfordjournals.jbchem.a021327;
Saguchi K., Tobe T., Hashimoto K., Nagasaki Y., Oda E., Nakano Y.,
Miura N.H., Tomita M.;
"Isolation and characterization of the human inter-alpha-trypsin
inhibitor family heavy chain-related protein (IHRP) gene (ITIHL1).";
J. Biochem. 119:898-905(1996).
[4]
NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 3).
TISSUE=Liver;
PubMed=14702039; DOI=10.1038/ng1285;
Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R.,
Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H.,
Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.,
Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K.,
Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A.,
Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M.,
Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y.,
Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M.,
Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K.,
Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S.,
Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J.,
Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y.,
Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N.,
Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S.,
Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S.,
Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O.,
Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H.,
Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B.,
Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y.,
Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T.,
Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y.,
Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S.,
Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T.,
Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M.,
Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T.,
Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K.,
Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R.,
Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.;
"Complete sequencing and characterization of 21,243 full-length human
cDNAs.";
Nat. Genet. 36:40-45(2004).
[5]
NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 4), AND INTERACTION WITH DNAJC1.
PubMed=16271702; DOI=10.1016/j.bbrc.2005.10.101;
Kroczynska B., King-Simmons L., Alloza L., Alava M.A., Elguindi E.C.,
Blond S.Y.;
"BIP co-chaperone MTJ1/ERDJ1 interacts with inter-alpha-trypsin
inhibitor heavy chain 4.";
Biochem. Biophys. Res. Commun. 338:1467-1477(2005).
[6]
NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
PubMed=16641997; DOI=10.1038/nature04728;
Muzny D.M., Scherer S.E., Kaul R., Wang J., Yu J., Sudbrak R.,
Buhay C.J., Chen R., Cree A., Ding Y., Dugan-Rocha S., Gill R.,
Gunaratne P., Harris R.A., Hawes A.C., Hernandez J., Hodgson A.V.,
Hume J., Jackson A., Khan Z.M., Kovar-Smith C., Lewis L.R.,
Lozado R.J., Metzker M.L., Milosavljevic A., Miner G.R., Morgan M.B.,
Nazareth L.V., Scott G., Sodergren E., Song X.-Z., Steffen D., Wei S.,
Wheeler D.A., Wright M.W., Worley K.C., Yuan Y., Zhang Z., Adams C.Q.,
Ansari-Lari M.A., Ayele M., Brown M.J., Chen G., Chen Z.,
Clendenning J., Clerc-Blankenburg K.P., Chen R., Chen Z., Davis C.,
Delgado O., Dinh H.H., Dong W., Draper H., Ernst S., Fu G.,
Gonzalez-Garay M.L., Garcia D.K., Gillett W., Gu J., Hao B.,
Haugen E., Havlak P., He X., Hennig S., Hu S., Huang W., Jackson L.R.,
Jacob L.S., Kelly S.H., Kube M., Levy R., Li Z., Liu B., Liu J.,
Liu W., Lu J., Maheshwari M., Nguyen B.-V., Okwuonu G.O., Palmeiri A.,
Pasternak S., Perez L.M., Phelps K.A., Plopper F.J., Qiang B.,
Raymond C., Rodriguez R., Saenphimmachak C., Santibanez J., Shen H.,
Shen Y., Subramanian S., Tabor P.E., Verduzco D., Waldron L., Wang J.,
Wang J., Wang Q., Williams G.A., Wong G.K.-S., Yao Z., Zhang J.,
Zhang X., Zhao G., Zhou J., Zhou Y., Nelson D., Lehrach H.,
Reinhardt R., Naylor S.L., Yang H., Olson M., Weinstock G.,
Gibbs R.A.;
"The DNA sequence, annotation and analysis of human chromosome 3.";
Nature 440:1194-1198(2006).
[7]
NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 271-930 (ISOFORM 2).
TISSUE=Fetal liver;
Zhang C., Yu Y., Zhang S., Wei H., Zhang Y., Zhou G., Bi J., Liu M.,
He F.;
"Functional prediction of the coding sequences of 79 new genes deduced
by analysis of cDNA clones from human fetal liver.";
Submitted (JAN-1999) to the EMBL/GenBank/DDBJ databases.
[8]
PROTEIN SEQUENCE OF 29-44; 99-119; 140-151; 163-170; 194-208; 211-243;
274-281; 290-329; 429-487; 497-567; 608-626 AND 816-831, PROTEOLYTIC
PROCESSING, AND GLYCOSYLATION.
TISSUE=Plasma;
PubMed=7541790; DOI=10.1093/jb/117.2.400;
Choi-Miura N.-H., Sano Y., Oda E., Nakano Y., Tobe T., Yanagishita T.,
Taniyama M., Katagiri T., Tomita M.;
"Purification and characterization of a novel glycoprotein which has
significant homology to heavy chains of inter-alpha-trypsin inhibitor
family from human plasma.";
J. Biochem. 117:400-407(1995).
[9]
PARTIAL PROTEIN SEQUENCE, AND PROTEOLYTIC PROCESSING.
PubMed=7947966; DOI=10.1016/0167-4838(94)90122-8;
Pu X.P., Iwamoto A., Nishimura H., Nagasawa S.;
"Purification and characterization of a novel substrate for plasma
kallikrein (PK-120) in human plasma.";
Biochim. Biophys. Acta 1208:338-343(1994).
[10]
IDENTIFICATION, AND INDUCTION.
PubMed=10486281; DOI=10.1006/bbrc.1999.1349;
Pineiro M., Alava M.A., Gonzalez-Ramon N., Osada J., Lasierra P.,
Larrad L., Pineiro A., Lampreave F.;
"ITIH4 serum concentration increases during acute-phase processes in
human patients and is up-regulated by interleukin-6 in hepatocarcinoma
HepG2 cells.";
Biochem. Biophys. Res. Commun. 263:224-229(1999).
[11]
GLYCOSYLATION AT ASN-207.
PubMed=12754519; DOI=10.1038/nbt827;
Zhang H., Li X.-J., Martin D.B., Aebersold R.;
"Identification and quantification of N-linked glycoproteins using
hydrazide chemistry, stable isotope labeling and mass spectrometry.";
Nat. Biotechnol. 21:660-666(2003).
[12]
GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-517.
TISSUE=Plasma;
PubMed=14760718; DOI=10.1002/pmic.200300556;
Bunkenborg J., Pilch B.J., Podtelejnikov A.V., Wisniewski J.R.;
"Screening for N-glycosylated proteins by liquid chromatography mass
spectrometry.";
Proteomics 4:454-465(2004).
[13]
GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-81; ASN-207; ASN-517 AND
ASN-577.
TISSUE=Plasma;
PubMed=16335952; DOI=10.1021/pr0502065;
Liu T., Qian W.-J., Gritsenko M.A., Camp D.G. II, Monroe M.E.,
Moore R.J., Smith R.D.;
"Human plasma N-glycoproteome analysis by immunoaffinity subtraction,
hydrazide chemistry, and mass spectrometry.";
J. Proteome Res. 4:2070-2080(2005).
[14]
FUNCTION, AND INDUCTION.
PubMed=19263524; DOI=10.1016/j.cca.2009.01.009;
Kashyap R.S., Nayak A.R., Deshpande P.S., Kabra D., Purohit H.J.,
Taori G.M., Daginawala H.F.;
"Inter-alpha-trypsin inhibitor heavy chain 4 is a novel marker of
acute ischemic stroke.";
Clin. Chim. Acta 402:160-163(2009).
[15]
GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-81; ASN-207 AND ASN-517.
TISSUE=Liver;
PubMed=19159218; DOI=10.1021/pr8008012;
Chen R., Jiang X., Sun D., Han G., Wang F., Ye M., Wang L., Zou H.;
"Glycoproteomics analysis of human liver tissue by combination of
multiple enzyme digestion and hydrazide chemistry.";
J. Proteome Res. 8:651-661(2009).
[16]
POSSIBLE ROLE OF ACTIVE PEPTIDE AS A BIOMARKER FOR BREAST CANCER.
PubMed=21137033; DOI=10.1002/prca.201000035;
van den Broek I., Sparidans R.W., van Winden A.W., Gast M.C.,
van Dulken E.J., Schellens J.H., Beijnen J.H.;
"The absolute quantification of eight inter-alpha-trypsin inhibitor
heavy chain 4 (ITIH4)-derived peptides in serum from breast cancer
patients.";
Proteomics Clin. Appl. 4:931-939(2010).
[17]
IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
PubMed=21269460; DOI=10.1186/1752-0509-5-17;
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P.,
Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.;
"Initial characterization of the human central proteome.";
BMC Syst. Biol. 5:17-17(2011).
[18]
GLYCOSYLATION AT THR-720, STRUCTURE OF CARBOHYDRATES, AND
IDENTIFICATION BY MASS SPECTROMETRY.
PubMed=22171320; DOI=10.1074/mcp.M111.013649;
Halim A., Nilsson J., Ruetschi U., Hesse C., Larson G.;
"Human urinary glycoproteomics; attachment site specific analysis of
N-and O-linked glycosylations by CID and ECD.";
Mol. Cell. Proteomics 0:0-0(2011).
[19]
GLYCOSYLATION AT ASN-81; ASN-207; ASN-274; ASN-517 AND ASN-577, AND
IDENTIFICATION BY MASS SPECTROMETRY.
PubMed=24884609; DOI=10.1021/pr500394z;
Chandler K.B., Brnakova Z., Sanda M., Wang S., Stalnaker S.H.,
Bridger R., Zhao P., Wells L., Edwards N.J., Goldman R.;
"Site-specific glycan microheterogeneity of inter-alpha-trypsin
inhibitor heavy chain H4.";
J. Proteome Res. 13:3314-3329(2014).
[20]
IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
TISSUE=Liver;
PubMed=24275569; DOI=10.1016/j.jprot.2013.11.014;
Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D.,
Wang L., Ye M., Zou H.;
"An enzyme assisted RP-RPLC approach for in-depth analysis of human
liver phosphoproteome.";
J. Proteomics 96:253-262(2014).
[21]
VARIANT ASN-86.
PubMed=12147176; DOI=10.1186/1471-2350-3-6;
Tozaki T., Choi-Miura N.-H., Taniyama M., Kurosawa M., Tomita M.;
"SNP analysis of the inter-alpha-trypsin inhibitor family heavy chain-
related protein (IHRP) gene by a fluorescence-adapted SSCP method.";
BMC Med. Genet. 3:6-6(2002).
-!- FUNCTION: Type II acute-phase protein (APP) involved in
inflammatory responses to trauma. May also play a role in liver
development or regeneration. {ECO:0000269|PubMed:19263524}.
-!- SUBUNIT: Interacts (via C-terminus) with DNAJC1 (via SANT 2
domain); this interaction protects ITIH4 against cleavage by
kallikrein in vitro. {ECO:0000269|PubMed:16271702}.
-!- SUBCELLULAR LOCATION: Secreted.
-!- ALTERNATIVE PRODUCTS:
Event=Alternative splicing; Named isoforms=4;
Name=1;
IsoId=Q14624-1; Sequence=Displayed;
Name=2;
IsoId=Q14624-2; Sequence=VSP_002761, VSP_002762;
Note=No experimental confirmation available.;
Name=3;
IsoId=Q14624-3; Sequence=VSP_002761;
Note=No experimental confirmation available.;
Name=4;
IsoId=Q14624-4; Sequence=VSP_002761, VSP_044764, VSP_044765;
Note=No experimental confirmation available.;
-!- TISSUE SPECIFICITY: Liver specific. {ECO:0000269|PubMed:7775381,
ECO:0000269|PubMed:7805892}.
-!- INDUCTION: Levels increase from 1.4 to 3-fold in acute-phase
processes such as in acute ischemia stroke (AIS), unstable angina
and programmed surgery. In hepatocytes, induced by IL6 but not by
other cytokines such as IL1B. {ECO:0000269|PubMed:10486281,
ECO:0000269|PubMed:19263524}.
-!- PTM: Cleaved by plasma kallikrein to yield 100 kDa and 35 kDa
fragments, and the resulting 100 kDa fragment is further converted
to a 70 kDa fragment.
-!- PTM: N- and O-glycosylated. In urine, O-linked glycosylation on
threonine residues in the region from Thr-719 to Thr-725 consists
of core 1 or possibly core 8 glycans. Mainly Hex(HexNAc)(2), but
also some Hex(3)(HexNAc)(3). N-glycosylated but not O-glycosylated
in plasma. {ECO:0000269|PubMed:12754519,
ECO:0000269|PubMed:14760718, ECO:0000269|PubMed:16335952,
ECO:0000269|PubMed:19159218, ECO:0000269|PubMed:22171320,
ECO:0000269|PubMed:7541790}.
-!- MISCELLANEOUS: Possible biomarker for acute ischemic stroke
(PubMed:19263524). Peptides derived from the proline-rich
potentially active peptide (PRO_0000016542) may be biomarkers for
a variety of disease states including breast cancer
(PubMed:21137033). {ECO:0000305|PubMed:19263524,
ECO:0000305|PubMed:21137033}.
-!- SIMILARITY: Belongs to the ITIH family. {ECO:0000305}.
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EMBL; D38535; BAA07536.1; -; mRNA.
EMBL; D38595; BAA07602.1; -; mRNA.
EMBL; U43163; AAD05198.1; -; Genomic_DNA.
EMBL; U42015; AAD05198.1; JOINED; Genomic_DNA.
EMBL; U42016; AAD05198.1; JOINED; Genomic_DNA.
EMBL; U43155; AAD05198.1; JOINED; Genomic_DNA.
EMBL; U43156; AAD05198.1; JOINED; Genomic_DNA.
EMBL; U43157; AAD05198.1; JOINED; Genomic_DNA.
EMBL; U43158; AAD05198.1; JOINED; Genomic_DNA.
EMBL; U43159; AAD05198.1; JOINED; Genomic_DNA.
EMBL; U43160; AAD05198.1; JOINED; Genomic_DNA.
EMBL; U43161; AAD05198.1; JOINED; Genomic_DNA.
EMBL; U43162; AAD05198.1; JOINED; Genomic_DNA.
EMBL; AK298412; BAH12781.1; -; mRNA.
EMBL; AC006254; -; NOT_ANNOTATED_CDS; Genomic_DNA.
EMBL; AC099667; -; NOT_ANNOTATED_CDS; Genomic_DNA.
EMBL; AF119856; AAF69610.1; -; mRNA.
CCDS; CCDS2865.1; -. [Q14624-1]
CCDS; CCDS54596.1; -. [Q14624-3]
PIR; JX0368; JX0368.
RefSeq; NP_001159921.1; NM_001166449.1. [Q14624-3]
RefSeq; NP_002209.2; NM_002218.4. [Q14624-1]
UniGene; Hs.709406; -.
ProteinModelPortal; Q14624; -.
BioGrid; 109906; 4.
IntAct; Q14624; 2.
STRING; 9606.ENSP00000266041; -.
iPTMnet; Q14624; -.
PhosphoSitePlus; Q14624; -.
BioMuta; ITIH4; -.
DMDM; 229463048; -.
PaxDb; Q14624; -.
PeptideAtlas; Q14624; -.
PRIDE; Q14624; -.
Ensembl; ENST00000266041; ENSP00000266041; ENSG00000055955. [Q14624-1]
Ensembl; ENST00000406595; ENSP00000384425; ENSG00000055955. [Q14624-3]
GeneID; 3700; -.
KEGG; hsa:3700; -.
UCSC; uc003dfz.4; human. [Q14624-1]
CTD; 3700; -.
DisGeNET; 3700; -.
EuPathDB; HostDB:ENSG00000055955.15; -.
GeneCards; ITIH4; -.
HGNC; HGNC:6169; ITIH4.
HPA; CAB072872; -.
HPA; HPA001835; -.
HPA; HPA003948; -.
MalaCards; ITIH4; -.
MIM; 600564; gene.
neXtProt; NX_Q14624; -.
OpenTargets; ENSG00000055955; -.
PharmGKB; PA29967; -.
eggNOG; ENOG410IEJB; Eukaryota.
eggNOG; COG2304; LUCA.
GeneTree; ENSGT00550000074468; -.
HOGENOM; HOG000000680; -.
HOVERGEN; HBG057734; -.
InParanoid; Q14624; -.
PhylomeDB; Q14624; -.
TreeFam; TF328982; -.
Reactome; R-HSA-114608; Platelet degranulation.
ChiTaRS; ITIH4; human.
GeneWiki; ITIH4; -.
GenomeRNAi; 3700; -.
PRO; PR:Q14624; -.
Proteomes; UP000005640; Chromosome 3.
Bgee; ENSG00000055955; -.
CleanEx; HS_ITIH4; -.
ExpressionAtlas; Q14624; baseline and differential.
Genevisible; Q14624; HS.
GO; GO:0072562; C:blood microparticle; IDA:UniProtKB.
GO; GO:0070062; C:extracellular exosome; IDA:UniProtKB.
GO; GO:0005576; C:extracellular region; TAS:Reactome.
GO; GO:0005886; C:plasma membrane; IEA:Ensembl.
GO; GO:0031089; C:platelet dense granule lumen; TAS:Reactome.
GO; GO:0004866; F:endopeptidase inhibitor activity; TAS:ProtInc.
GO; GO:0004867; F:serine-type endopeptidase inhibitor activity; IEA:UniProtKB-KW.
GO; GO:0006953; P:acute-phase response; IEP:UniProtKB.
GO; GO:0030212; P:hyaluronan metabolic process; IEA:InterPro.
GO; GO:0002576; P:platelet degranulation; TAS:Reactome.
GO; GO:0034097; P:response to cytokine; IEP:UniProtKB.
Gene3D; 3.40.50.410; -; 1.
InterPro; IPR010600; ITI_HC_C.
InterPro; IPR013694; VIT.
InterPro; IPR002035; VWF_A.
InterPro; IPR036465; vWFA_dom_sf.
Pfam; PF06668; ITI_HC_C; 1.
Pfam; PF08487; VIT; 1.
Pfam; PF00092; VWA; 1.
SMART; SM00609; VIT; 1.
SMART; SM00327; VWA; 1.
SUPFAM; SSF53300; SSF53300; 1.
PROSITE; PS51468; VIT; 1.
PROSITE; PS50234; VWFA; 1.
1: Evidence at protein level;
Acute phase; Alternative splicing; Complete proteome;
Direct protein sequencing; Disulfide bond; Glycoprotein; Polymorphism;
Protease inhibitor; Reference proteome; Secreted;
Serine protease inhibitor; Signal.
SIGNAL 1 28 {ECO:0000269|PubMed:7541790}.
CHAIN 29 661 70 kDa inter-alpha-trypsin inhibitor
heavy chain H4.
/FTId=PRO_0000016541.
PROPEP 662 688 Potentially active peptide.
/FTId=PRO_0000016542.
CHAIN 689 930 35 kDa inter-alpha-trypsin inhibitor
heavy chain H4.
/FTId=PRO_0000016543.
DOMAIN 29 148 VIT. {ECO:0000255|PROSITE-
ProRule:PRU00801}.
DOMAIN 272 432 VWFA. {ECO:0000255|PROSITE-
ProRule:PRU00219}.
REGION 658 688 Proline-rich (PRR) potential bioactive
peptide.
REGION 719 725 O-glycosylated at three sites.
SITE 688 689 Cleavage; by kallikrein.
CARBOHYD 81 81 N-linked (GlcNAc...) asparagine.
{ECO:0000269|PubMed:16335952,
ECO:0000269|PubMed:19159218,
ECO:0000269|PubMed:24884609}.
CARBOHYD 207 207 N-linked (GlcNAc...) asparagine.
{ECO:0000269|PubMed:12754519,
ECO:0000269|PubMed:16335952,
ECO:0000269|PubMed:19159218,
ECO:0000269|PubMed:24884609}.
CARBOHYD 274 274 N-linked (GlcNAc...) asparagine;
atypical. {ECO:0000269|PubMed:24884609}.
CARBOHYD 517 517 N-linked (GlcNAc...) asparagine.
{ECO:0000269|PubMed:14760718,
ECO:0000269|PubMed:16335952,
ECO:0000269|PubMed:19159218,
ECO:0000269|PubMed:24884609}.
CARBOHYD 577 577 N-linked (GlcNAc...) asparagine.
{ECO:0000269|PubMed:16335952,
ECO:0000269|PubMed:24884609}.
CARBOHYD 719 719 O-linked (GalNAc...) threonine.
{ECO:0000250|UniProtKB:Q3T052}.
CARBOHYD 720 720 O-linked (GalNAc...) threonine.
{ECO:0000269|PubMed:22171320}.
CARBOHYD 722 722 O-linked (GalNAc...) threonine.
{ECO:0000250|UniProtKB:Q3T052}.
DISULFID 747 925 {ECO:0000305}.
VAR_SEQ 621 650 Missing (in isoform 2, isoform 3 and
isoform 4). {ECO:0000303|PubMed:14702039,
ECO:0000303|PubMed:16271702,
ECO:0000303|Ref.7}.
/FTId=VSP_002761.
VAR_SEQ 727 765 Missing (in isoform 4).
{ECO:0000303|PubMed:16271702}.
/FTId=VSP_044764.
VAR_SEQ 727 727 A -> ACPSCSRSRAPAVPA (in isoform 2).
{ECO:0000303|Ref.7}.
/FTId=VSP_002762.
VAR_SEQ 851 866 Missing (in isoform 4).
{ECO:0000303|PubMed:16271702}.
/FTId=VSP_044765.
VARIANT 85 85 I -> N (in dbSNP:rs13072536).
{ECO:0000269|PubMed:7775381}.
/FTId=VAR_027869.
VARIANT 86 86 I -> N. {ECO:0000269|PubMed:12147176}.
/FTId=VAR_013836.
VARIANT 669 669 Q -> L (in dbSNP:rs2276814).
/FTId=VAR_027870.
VARIANT 698 698 P -> T (in dbSNP:rs4687657).
/FTId=VAR_027871.
VARIANT 714 714 M -> I (in dbSNP:rs2256734).
/FTId=VAR_027872.
VARIANT 791 791 L -> P (in dbSNP:rs2535621).
/FTId=VAR_027873.
CONFLICT 85 85 I -> K (in Ref. 3; AAD05198).
{ECO:0000305}.
CONFLICT 114 114 S -> N (in Ref. 2; BAA07602).
{ECO:0000305}.
CONFLICT 207 207 N -> F (in Ref. 8; AA sequence).
{ECO:0000305}.
CONFLICT 221 221 Q -> E (in Ref. 8; AA sequence).
{ECO:0000305}.
CONFLICT 307 307 R -> V (in Ref. 8; AA sequence).
{ECO:0000305}.
CONFLICT 322 322 W -> Y (in Ref. 8; AA sequence).
{ECO:0000305}.
CONFLICT 370 370 E -> G (in Ref. 6; BAH12781).
{ECO:0000305}.
CONFLICT 816 817 ET -> QR (in Ref. 8; AA sequence).
{ECO:0000305}.
CONFLICT 905 905 S -> F (in Ref. 3; no nucleotide entry).
{ECO:0000305}.
CONFLICT 927 927 S -> T (in Ref. 3; no nucleotide entry).
{ECO:0000305}.
SEQUENCE 930 AA; 103357 MW; 847A9A5CB886C5A4 CRC64;
MKPPRPVRTC SKVLVLLSLL AIHQTTTAEK NGIDIYSLTV DSRVSSRFAH TVVTSRVVNR
ANTVQEATFQ MELPKKAFIT NFSMIIDGMT YPGIIKEKAE AQAQYSAAVA KGKSAGLVKA
TGRNMEQFQV SVSVAPNAKI TFELVYEELL KRRLGVYELL LKVRPQQLVK HLQMDIHIFE
PQGISFLETE STFMTNQLVD ALTTWQNKTK AHIRFKPTLS QQQKSPEQQE TVLDGNLIIR
YDVDRAISGG SIQIENGYFV HYFAPEGLTT MPKNVVFVID KSGSMSGRKI QQTREALIKI
LDDLSPRDQF NLIVFSTEAT QWRPSLVPAS AENVNKARSF AAGIQALGGT NINDAMLMAV
QLLDSSNQEE RLPEGSVSLI ILLTDGDPTV GETNPRSIQN NVREAVSGRY SLFCLGFGFD
VSYAFLEKLA LDNGGLARRI HEDSDSALQL QDFYQEVANP LLTAVTFEYP SNAVEEVTQN
NFRLLFKGSE MVVAGKLQDR GPDVLTATVS GKLPTQNITF QTESSVAEQE AEFQSPKYIF
HNFMERLWAY LTIQQLLEQT VSASDADQQA LRNQALNLSL AYSFVTPLTS MVVTKPDDQE
QSQVAEKPME GESRNRNVHS GSTFFKYYLQ GAKIPKPEAS FSPRRGWNRQ AGAAGSRMNF
RPGVLSSRQL GLPGPPDVPD HAAYHPFRRL AILPASAPPA TSNPDPAVSR VMNMKIEETT
MTTQTPAPIQ APSAILPLPG QSVERLCVDP RHRQGPVNLL SDPEQGVEVT GQYEREKAGF
SWIEVTFKNP LVWVHASPEH VVVTRNRRSS AYKWKETLFS VMPGLKMTMD KTGLLLLSDP
DKVTIGLLFW DGRGEGLRLL LRDTDRFSSH VGGTLGQFYQ EVLWGSPAAS DDGRRTLRVQ
GNDHSATRER RLDYQEGPPG VEISCWSVEL


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