UniProtKB - P19827 (ITIH1_HUMAN)
Inter-alpha-trypsin inhibitor heavy chain H1
ITIH1
Functioni
May act as a carrier of hyaluronan in serum or as a binding protein between hyaluronan and other matrix protein, including those on cell surfaces in tissues to regulate the localization, synthesis and degradation of hyaluronan which are essential to cells undergoing biological processes.
Contains a potential peptide which could stimulate a broad spectrum of phagocytotic cells.
GO - Molecular functioni
- calcium ion binding Source: ProtInc
- hyaluronic acid binding Source: UniProtKB
- serine-type endopeptidase inhibitor activity Source: UniProtKB-KW
GO - Biological processi
- hyaluronan metabolic process Source: InterPro
Keywordsi
Molecular function | Protease inhibitor, Serine protease inhibitor |
Enzyme and pathway databases
PathwayCommonsi | P19827 |
SignaLinki | P19827 |
Names & Taxonomyi
Protein namesi | Recommended name: Inter-alpha-trypsin inhibitor heavy chain H1Short name: ITI heavy chain H1 Short name: ITI-HC1 Short name: Inter-alpha-inhibitor heavy chain 1 Alternative name(s): Inter-alpha-trypsin inhibitor complex component III Serum-derived hyaluronan-associated protein Short name: SHAP |
Gene namesi | Name:ITIH1 Synonyms:IGHEP1 |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:6166, ITIH1 |
MIMi | 147270, gene |
neXtProti | NX_P19827 |
VEuPathDBi | HostDB:ENSG00000055957 |
Subcellular locationi
Extracellular region or secreted
Extracellular region or secreted
- blood microparticle Source: UniProtKB
- extracellular exosome Source: UniProtKB
- extracellular region Source: UniProtKB
Other locations
- collagen-containing extracellular matrix Source: BHF-UCL
Keywords - Cellular componenti
SecretedPathology & Biotechi
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 298 | D → A: Abolishes binding to CUB domain of TNFAIP6. 1 Publication | 1 |
Organism-specific databases
DisGeNETi | 3697 |
OpenTargetsi | ENSG00000055957 |
PharmGKBi | PA29964 |
Miscellaneous databases
Pharosi | P19827, Tbio |
Chemistry databases
DrugBanki | DB09338, Mersalyl DB01593, Zinc DB14487, Zinc acetate DB14533, Zinc chloride DB14548, Zinc sulfate, unspecified form |
Genetic variation databases
BioMutai | ITIH1 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Signal peptidei | 1 – 27 | Sequence analysisAdd BLAST | 27 | |
PropeptideiPRO_0000016506 | 28 – 34 | 1 Publication | 7 | |
ChainiPRO_0000016507 | 35 – 672 | Inter-alpha-trypsin inhibitor heavy chain H1Add BLAST | 638 | |
PropeptideiPRO_0000016508 | 673 – 911 | Add BLAST | 239 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Glycosylationi | 60 | S-linked (Hex...) cysteine1 Publication | 1 | |
Modified residuei | 129 | PhosphoserineCombined sources | 1 | |
Disulfide bondi | 244 ↔ 247 | 1 Publication | ||
Disulfide bondi | 268 ↔ 540 | 1 Publication | ||
GlycosylationiCAR_000138 | 285 | N-linked (GlcNAc...) (complex) asparagine4 Publications | 1 | |
Modified residuei | 402 | PhosphothreonineCombined sources | 1 | |
Modified residuei | 407 | PhosphothreonineCombined sources | 1 | |
GlycosylationiCAR_000139 | 588 | N-linked (GlcNAc...) (complex) asparagine3 Publications | 1 | |
GlycosylationiCAR_000213 | 653 | O-linked (GalNAc...) threonine1 Publication | 1 | |
Modified residuei | 672 | Aspartate 1-(chondroitin 4-sulfate)-ester | 1 | |
Glycosylationi | 750 | N-linked (GlcNAc...) asparagine1 Publication | 1 |
Post-translational modificationi
Keywords - PTMi
Disulfide bond, Glycoprotein, Phosphoprotein, ProteoglycanProteomic databases
EPDi | P19827 |
jPOSTi | P19827 |
MassIVEi | P19827 |
PaxDbi | P19827 |
PeptideAtlasi | P19827 |
PRIDEi | P19827 |
ProteomicsDBi | 25555 27630 53691 [P19827-1] |
PTM databases
GlyConnecti | 284, 17 N-Linked glycans (2 sites), 1 O-Linked glycan (1 site) |
GlyGeni | P19827, 8 sites, 26 N-linked glycans (3 sites), 3 O-linked glycans (3 sites) |
iPTMneti | P19827 |
PhosphoSitePlusi | P19827 |
Expressioni
Gene expression databases
Bgeei | ENSG00000055957, Expressed in right lobe of liver and 114 other tissues |
ExpressionAtlasi | P19827, baseline and differential |
Genevisiblei | P19827, HS |
Organism-specific databases
HPAi | ENSG00000055957, Tissue enriched (liver) |
Interactioni
Subunit structurei
Protein-protein interaction databases
BioGRIDi | 109903, 21 interactors |
IntActi | P19827, 4 interactors |
STRINGi | 9606.ENSP00000273283 |
Miscellaneous databases
RNActi | P19827, protein |
Structurei
Secondary structure
3D structure databases
AlphaFoldDBi | P19827 |
SMRi | P19827 |
ModBasei | Search... |
PDBe-KBi | Search... |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 37 – 166 | VITPROSITE-ProRule annotationAdd BLAST | 130 | |
Domaini | 290 – 450 | VWFAPROSITE-ProRule annotationAdd BLAST | 161 |
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 387 – 911 | Hyaluronan-bindingAdd BLAST | 525 |
Motif
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Motifi | 181 – 184 | Phagocytosis uptake signalSequence analysis | 4 |
Sequence similaritiesi
Keywords - Domaini
SignalPhylogenomic databases
eggNOGi | ENOG502RXR2, Eukaryota |
GeneTreei | ENSGT00940000162180 |
HOGENOMi | CLU_008101_0_0_1 |
InParanoidi | P19827 |
OMAi | FNFLEAM |
OrthoDBi | 955432at2759 |
PhylomeDBi | P19827 |
TreeFami | TF328982 |
Family and domain databases
Gene3Di | 3.40.50.410, 1 hit |
InterProi | View protein in InterPro IPR010600, ITI_HC_C IPR013694, VIT IPR002035, VWF_A IPR036465, vWFA_dom_sf |
Pfami | View protein in Pfam PF06668, ITI_HC_C, 1 hit PF08487, VIT, 1 hit PF00092, VWA, 1 hit |
SMARTi | View protein in SMART SM00609, VIT, 1 hit SM00327, VWA, 1 hit |
SUPFAMi | SSF53300, SSF53300, 1 hit |
PROSITEi | View protein in PROSITE PS51468, VIT, 1 hit PS50234, VWFA, 1 hit |
s (3+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 3 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 3 described isoforms and 4 potential isoforms that are computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MDGAMGPRGL LLCMYLVSLL ILQAMPALGS ATGRSKSSEK RQAVDTAVDG
60 70 80 90 100
VFIRSLKVNC KVTSRFAHYV VTSQVVNTAN EAREVAFDLE IPKTAFISDF
110 120 130 140 150
AVTADGNAFI GDIKDKVTAW KQYRKAAISG ENAGLVRASG RTMEQFTIHL
160 170 180 190 200
TVNPQSKVTF QLTYEEVLKR NHMQYEIVIK VKPKQLVHHF EIDVDIFEPQ
210 220 230 240 250
GISKLDAQAS FLPKELAAQT IKKSFSGKKG HVLFRPTVSQ QQSCPTCSTS
260 270 280 290 300
LLNGHFKVTY DVSRDKICDL LVANNHFAHF FAPQNLTNMN KNVVFVIDIS
310 320 330 340 350
GSMRGQKVKQ TKEALLKILG DMQPGDYFDL VLFGTRVQSW KGSLVQASEA
360 370 380 390 400
NLQAAQDFVR GFSLDEATNL NGGLLRGIEI LNQVQESLPE LSNHASILIM
410 420 430 440 450
LTDGDPTEGV TDRSQILKNV RNAIRGRFPL YNLGFGHNVD FNFLEVMSME
460 470 480 490 500
NNGRAQRIYE DHDATQQLQG FYSQVAKPLL VDVDLQYPQD AVLALTQNHH
510 520 530 540 550
KQYYEGSEIV VAGRIADNKQ SSFKADVQAH GEGQEFSITC LVDEEEMKKL
560 570 580 590 600
LRERGHMLEN HVERLWAYLT IQELLAKRMK VDREERANLS SQALQMSLDY
610 620 630 640 650
GFVTPLTSMS IRGMADQDGL KPTIDKPSED SPPLEMLGPR RTFVLSALQP
660 670 680 690 700
SPTHSSSNTQ RLPDRVTGVD TDPHFIIHVP QKEDTLCFNI NEEPGVILSL
710 720 730 740 750
VQDPNTGFSV NGQLIGNKAR SPGQHDGTYF GRLGIANPAT DFQLEVTPQN
760 770 780 790 800
ITLNPGFGGP VFSWRDQAVL RQDGVVVTIN KKRNLVVSVD DGGTFEVVLH
810 820 830 840 850
RVWKGSSVHQ DFLGFYVLDS HRMSARTHGL LGQFFHPIGF EVSDIHPGSD
860 870 880 890 900
PTKPDATMVV RNRRLTVTRG LQKDYSKDPW HGAEVSCWFI HNNGAGLIDG
910
AYTDYIVPDI F
Computationally mapped potential isoform sequencesi
There are 4 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketB5MCP1 | B5MCP1_HUMAN | Inter-alpha-trypsin inhibitor heavy... | ITIH1 | 277 | Annotation score: | ||
F8WAS2 | F8WAS2_HUMAN | Inter-alpha-trypsin inhibitor heavy... | ITIH1 | 213 | Annotation score: | ||
H7C0N0 | H7C0N0_HUMAN | Inter-alpha-trypsin inhibitor heavy... | ITIH1 | 464 | Annotation score: | ||
H7C5I0 | H7C5I0_HUMAN | Inter-alpha-trypsin inhibitor heavy... | ITIH1 | 74 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 51 | V → T AA sequence (PubMed:2476436).Curated | 1 | |
Sequence conflicti | 54 | R → A AA sequence (PubMed:2476436).Curated | 1 | |
Sequence conflicti | 266 | K → E in CAA45188 (PubMed:1380832).Curated | 1 | |
Sequence conflicti | 539 | T → A in BAH12794 (PubMed:14702039).Curated | 1 | |
Sequence conflicti | 798 | V → A in BAH12794 (PubMed:14702039).Curated | 1 |
Mass spectrometryi
Polymorphismi
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_011873 | 263 | S → T1 PublicationCorresponds to variant dbSNP:rs1042777Ensembl. | 1 | |
Natural variantiVAR_004019 | 585 | E → V in allele ITIH1*2. 3 PublicationsCorresponds to variant dbSNP:rs678EnsemblClinVar. | 1 | |
Natural variantiVAR_004020 | 595 | Q → R in allele ITIH1*2 and allele ITIH1*3. 3 PublicationsCorresponds to variant dbSNP:rs1042779EnsemblClinVar. | 1 | |
Natural variantiVAR_011874 | 695 | G → C. Corresponds to variant dbSNP:rs1042904Ensembl. | 1 | |
Natural variantiVAR_011875 | 844 | D → E. Corresponds to variant dbSNP:rs1042849Ensembl. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_045419 | 1 – 288 | Missing in isoform 3. 1 PublicationAdd BLAST | 288 | |
Alternative sequenceiVSP_045420 | 1 – 142 | Missing in isoform 2. 1 PublicationAdd BLAST | 142 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | X63652 mRNA Translation: CAA45188.1 X69532 , X69533, X69534, X69535, X69536, X69537, X69538, X69539, X69540, X69541, X69542, X69543, X69544, X69545, X69546, X69547 Genomic DNA Translation: CAA49279.1 AK292750 mRNA Translation: BAF85439.1 AK298455 mRNA Translation: BAH12794.1 AK303156 mRNA Translation: BAH13906.1 AK314198 mRNA Translation: BAG36876.1 AC006254 Genomic DNA No translation available. CH471055 Genomic DNA Translation: EAW65259.1 BC069464 mRNA Translation: AAH69464.1 X16260 mRNA Translation: CAA34346.1 M18192 mRNA Translation: AAA60557.1 X75318 Genomic DNA Translation: CAA53067.1 |
CCDSi | CCDS2864.1 [P19827-1] CCDS54595.1 [P19827-3] |
PIRi | S39527, A39967 |
RefSeqi | NP_001159906.1, NM_001166434.2 [P19827-2] NP_001159907.1, NM_001166435.2 [P19827-3] NP_002206.2, NM_002215.3 [P19827-1] |
Genome annotation databases
Ensembli | ENST00000273283.7; ENSP00000273283.2; ENSG00000055957.11 ENST00000537050.5; ENSP00000443847.1; ENSG00000055957.11 [P19827-3] |
GeneIDi | 3697 |
KEGGi | hsa:3697 |
MANE-Selecti | ENST00000273283.7; ENSP00000273283.2; NM_002215.4; NP_002206.2 |
UCSCi | uc003dfs.4, human [P19827-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | X63652 mRNA Translation: CAA45188.1 X69532 , X69533, X69534, X69535, X69536, X69537, X69538, X69539, X69540, X69541, X69542, X69543, X69544, X69545, X69546, X69547 Genomic DNA Translation: CAA49279.1 AK292750 mRNA Translation: BAF85439.1 AK298455 mRNA Translation: BAH12794.1 AK303156 mRNA Translation: BAH13906.1 AK314198 mRNA Translation: BAG36876.1 AC006254 Genomic DNA No translation available. CH471055 Genomic DNA Translation: EAW65259.1 BC069464 mRNA Translation: AAH69464.1 X16260 mRNA Translation: CAA34346.1 M18192 mRNA Translation: AAA60557.1 X75318 Genomic DNA Translation: CAA53067.1 |
CCDSi | CCDS2864.1 [P19827-1] CCDS54595.1 [P19827-3] |
PIRi | S39527, A39967 |
RefSeqi | NP_001159906.1, NM_001166434.2 [P19827-2] NP_001159907.1, NM_001166435.2 [P19827-3] NP_002206.2, NM_002215.3 [P19827-1] |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
6FPY | X-ray | 2.34 | A/B | 35-672 | [»] | |
6FPZ | X-ray | 2.20 | A/B | 35-672 | [»] | |
AlphaFoldDBi | P19827 | |||||
SMRi | P19827 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 109903, 21 interactors |
IntActi | P19827, 4 interactors |
STRINGi | 9606.ENSP00000273283 |
Chemistry databases
DrugBanki | DB09338, Mersalyl DB01593, Zinc DB14487, Zinc acetate DB14533, Zinc chloride DB14548, Zinc sulfate, unspecified form |
PTM databases
GlyConnecti | 284, 17 N-Linked glycans (2 sites), 1 O-Linked glycan (1 site) |
GlyGeni | P19827, 8 sites, 26 N-linked glycans (3 sites), 3 O-linked glycans (3 sites) |
iPTMneti | P19827 |
PhosphoSitePlusi | P19827 |
Genetic variation databases
BioMutai | ITIH1 |
Proteomic databases
EPDi | P19827 |
jPOSTi | P19827 |
MassIVEi | P19827 |
PaxDbi | P19827 |
PeptideAtlasi | P19827 |
PRIDEi | P19827 |
ProteomicsDBi | 25555 27630 53691 [P19827-1] |
Protocols and materials databases
Antibodypediai | 31337, 278 antibodies from 24 providers |
DNASUi | 3697 |
Genome annotation databases
Ensembli | ENST00000273283.7; ENSP00000273283.2; ENSG00000055957.11 ENST00000537050.5; ENSP00000443847.1; ENSG00000055957.11 [P19827-3] |
GeneIDi | 3697 |
KEGGi | hsa:3697 |
MANE-Selecti | ENST00000273283.7; ENSP00000273283.2; NM_002215.4; NP_002206.2 |
UCSCi | uc003dfs.4, human [P19827-1] |
Organism-specific databases
CTDi | 3697 |
DisGeNETi | 3697 |
GeneCardsi | ITIH1 |
HGNCi | HGNC:6166, ITIH1 |
HPAi | ENSG00000055957, Tissue enriched (liver) |
MIMi | 147270, gene |
neXtProti | NX_P19827 |
OpenTargetsi | ENSG00000055957 |
PharmGKBi | PA29964 |
VEuPathDBi | HostDB:ENSG00000055957 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | ENOG502RXR2, Eukaryota |
GeneTreei | ENSGT00940000162180 |
HOGENOMi | CLU_008101_0_0_1 |
InParanoidi | P19827 |
OMAi | FNFLEAM |
OrthoDBi | 955432at2759 |
PhylomeDBi | P19827 |
TreeFami | TF328982 |
Enzyme and pathway databases
PathwayCommonsi | P19827 |
SignaLinki | P19827 |
Miscellaneous databases
BioGRID-ORCSi | 3697, 11 hits in 1070 CRISPR screens |
ChiTaRSi | ITIH1, human |
GeneWikii | ITIH1 |
GenomeRNAii | 3697 |
Pharosi | P19827, Tbio |
PROi | PR:P19827 |
RNActi | P19827, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000055957, Expressed in right lobe of liver and 114 other tissues |
ExpressionAtlasi | P19827, baseline and differential |
Genevisiblei | P19827, HS |
Family and domain databases
Gene3Di | 3.40.50.410, 1 hit |
InterProi | View protein in InterPro IPR010600, ITI_HC_C IPR013694, VIT IPR002035, VWF_A IPR036465, vWFA_dom_sf |
Pfami | View protein in Pfam PF06668, ITI_HC_C, 1 hit PF08487, VIT, 1 hit PF00092, VWA, 1 hit |
SMARTi | View protein in SMART SM00609, VIT, 1 hit SM00327, VWA, 1 hit |
SUPFAMi | SSF53300, SSF53300, 1 hit |
PROSITEi | View protein in PROSITE PS51468, VIT, 1 hit PS50234, VWFA, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | ITIH1_HUMAN | |
Accessioni | P19827Primary (citable) accession number: P19827 Secondary accession number(s): A8K9N5 Q562G1 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | February 1, 1991 |
Last sequence update: | July 15, 1998 | |
Last modified: | May 25, 2022 | |
This is version 201 of the entry and version 3 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. |
Miscellaneousi
Keywords - Technical termi
3D-structure, Direct protein sequencing, Reference proteomeDocuments
- Human chromosome 3
Human chromosome 3: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families