UniProtKB - Q06033 (ITIH3_HUMAN)
Inter-alpha-trypsin inhibitor heavy chain H3
ITIH3
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.
GO - Molecular functioni
- endopeptidase inhibitor activity Source: ProtInc
- 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 | Q06033 |
Reactomei | R-HSA-114608, Platelet degranulation |
SignaLinki | Q06033 |
Names & Taxonomyi
Protein namesi | Recommended name: Inter-alpha-trypsin inhibitor heavy chain H3Short name: ITI heavy chain H3 Short name: ITI-HC3 Short name: Inter-alpha-inhibitor heavy chain 3 Alternative name(s): Serum-derived hyaluronan-associated protein Short name: SHAP |
Gene namesi | Name:ITIH3 |
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:6168, ITIH3 |
MIMi | 146650, gene |
neXtProti | NX_Q06033 |
VEuPathDBi | HostDB:ENSG00000162267 |
Subcellular locationi
Extracellular region or secreted
Extracellular region or secreted
- extracellular exosome Source: UniProtKB
- extracellular region Source: Reactome
Other locations
- platelet dense granule lumen Source: Reactome
Keywords - Cellular componenti
SecretedPathology & Biotechi
Organism-specific databases
DisGeNETi | 3699 |
OpenTargetsi | ENSG00000162267 |
PharmGKBi | PA29966 |
Miscellaneous databases
Pharosi | Q06033, Tbio |
Chemistry databases
DrugBanki | DB01593, Zinc DB14487, Zinc acetate DB14533, Zinc chloride DB14548, Zinc sulfate, unspecified form |
Genetic variation databases
BioMutai | ITIH3 |
DMDMi | 166203665 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Signal peptidei | 1 – 20 | Sequence analysisAdd BLAST | 20 | |
PropeptideiPRO_0000016529 | 21 – 34 | Add BLAST | 14 | |
ChainiPRO_0000016530 | 35 – 651 | Inter-alpha-trypsin inhibitor heavy chain H3Add BLAST | 617 | |
PropeptideiPRO_0000016531 | 652 – 890 | Add BLAST | 239 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Glycosylationi | 91 | N-linked (GlcNAc...) asparagine1 Publication | 1 | |
Glycosylationi | 580 | N-linked (GlcNAc...) asparagine1 Publication | 1 | |
Modified residuei | 651 | Aspartate 1-(chondroitin 4-sulfate)-ester | 1 |
Post-translational modificationi
Keywords - PTMi
Glycoprotein, ProteoglycanProteomic databases
jPOSTi | Q06033 |
MassIVEi | Q06033 |
MaxQBi | Q06033 |
PaxDbi | Q06033 |
PeptideAtlasi | Q06033 |
PRIDEi | Q06033 |
ProteomicsDBi | 58409 [Q06033-1] 58410 [Q06033-2] |
PTM databases
CarbonylDBi | Q06033 |
GlyConnecti | 1421, 10 N-Linked glycans (1 site) |
GlyGeni | Q06033, 2 sites, 10 N-linked glycans (1 site) |
iPTMneti | Q06033 |
PhosphoSitePlusi | Q06033 |
Expressioni
Gene expression databases
Bgeei | ENSG00000162267, Expressed in right lobe of liver and 131 other tissues |
ExpressionAtlasi | Q06033, baseline and differential |
Genevisiblei | Q06033, HS |
Organism-specific databases
HPAi | ENSG00000162267, Tissue enriched (liver) |
Interactioni
Subunit structurei
I-alpha-I plasma protease inhibitors are assembled from one or two heavy chains (HC) and one light chain, bikunin. Pre-alpha-inhibitor (P-alpha-I) is composed of ITIH3/HC3 and bikunin.
1 PublicationProtein-protein interaction databases
BioGRIDi | 109905, 17 interactors |
IntActi | Q06033, 7 interactors |
MINTi | Q06033 |
STRINGi | 9606.ENSP00000415769 |
Miscellaneous databases
RNActi | Q06033, protein |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 29 – 158 | VITPROSITE-ProRule annotationAdd BLAST | 130 | |
Domaini | 284 – 467 | VWFAPROSITE-ProRule annotationAdd BLAST | 184 |
Sequence similaritiesi
Keywords - Domaini
SignalPhylogenomic databases
eggNOGi | ENOG502QPS2, Eukaryota |
GeneTreei | ENSGT00940000154554 |
InParanoidi | Q06033 |
OMAi | EFIYWIN |
OrthoDBi | 955432at2759 |
PhylomeDBi | Q06033 |
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 (2+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 2 described isoforms and 2 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
MAFAWWPCLI LALLSSLAAS GFPRSPFRLL GKRSLPEGVA NGIEVYSTKI
60 70 80 90 100
NSKVTSRFAH NVVTMRAVNR ADTAKEVSFD VELPKTAFIT NFTLTIDGVT
110 120 130 140 150
YPGNVKEKEV AKKQYEKAVS QGKTAGLVKA SGRKLEKFTV SVNVAAGSKV
160 170 180 190 200
TFELTYEELL KRHKGKYEMY LKVQPKQLVK HFEIEVDIFE PQGISMLDAE
210 220 230 240 250
ASFITNDLLG SALTKSFSGK KGHVSFKPSL DQQRSCPTCT DSLLNGDFTI
260 270 280 290 300
TYDVNRESPG NVQIVNGYFV HFFAPQGLPV VPKNVAFVID ISGSMAGRKL
310 320 330 340 350
EQTKEALLRI LEDMQEEDYL NFILFSGDVS TWKEHLVQAT PENLQEARTF
360 370 380 390 400
VKSMEDKGMT NINDGLLRGI SMLNKAREEH RIPERSTSIV IMLTDGDANV
410 420 430 440 450
GESRPEKIQE NVRNAIGGKF PLYNLGFGNN LNYNFLENMA LENHGFARRI
460 470 480 490 500
YEDSDADLQL QGFYEEVANP LLTGVEMEYP ENAILDLTQN TYQHFYDGSE
510 520 530 540 550
IVVAGRLVDE DMNSFKADVK GHGATNDLTF TEEVDMKEME KALQERDYIF
560 570 580 590 600
GNYIERLWAY LTIEQLLEKR KNAHGEEKEN LTARALDLSL KYHFVTPLTS
610 620 630 640 650
MVVTKPEDNE DERAIADKPG EDAEATPVSP AMSYLTSYQP PQNPYYYVDG
660 670 680 690 700
DPHFIIQIPE KDDALCFNID EAPGTVLRLI QDAVTGLTVN GQITGDKRGS
710 720 730 740 750
PDSKTRKTYF GKLGIANAQM DFQVEVTTEK ITLWNRAVPS TFSWLDTVTV
760 770 780 790 800
TQDGLSMMIN RKNMVVSFGD GVTFVVVLHQ VWKKHPVHRD FLGFYVVDSH
810 820 830 840 850
RMSAQTHGLL GQFFQPFDFK VSDIRPGSDP TKPDATLVVK NHQLIVTRGS
860 870 880 890
QKDYRKDASI GTKVVCWFVH NNGEGLIDGV HTDYIVPNLF
Computationally mapped potential isoform sequencesi
There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketE7ET33 | E7ET33_HUMAN | Inter-alpha-trypsin inhibitor heavy... | ITIH3 | 698 | Annotation score: | ||
A0A087WW43 | A0A087WW43_HUMAN | Inter-alpha-trypsin inhibitor heavy... | ITIH3 | 670 | Annotation score: |
Sequence cautioni
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 113 | K → N in BAD97203 (Ref. 3) Curated | 1 | |
Sequence conflicti | 113 | K → N in BAD96477 (Ref. 3) Curated | 1 | |
Sequence conflicti | 348 | R → K in CAA32821 (PubMed:2465147).Curated | 1 | |
Sequence conflicti | 361 | N → G in CAA32821 (PubMed:2465147).Curated | 1 | |
Sequence conflicti | 515 | F → L in CAC79611 (PubMed:9756925).Curated | 1 | |
Sequence conflicti | 667 | F → L in BAD97203 (Ref. 3) Curated | 1 | |
Sequence conflicti | 667 | F → L in BAD96477 (Ref. 3) Curated | 1 | |
Sequence conflicti | 716 | A → R in CAA47439 (PubMed:7681778).Curated | 1 | |
Sequence conflicti | 716 | A → R in CAA32821 (PubMed:2465147).Curated | 1 | |
Sequence conflicti | 851 | Q → H in CAA32821 (PubMed:2465147).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_049647 | 315 | Q → K1 PublicationCorresponds to variant dbSNP:rs3617Ensembl. | 1 | |
Natural variantiVAR_049648 | 340 | T → M. Corresponds to variant dbSNP:rs35271262Ensembl. | 1 | |
Natural variantiVAR_061275 | 640 | P → L. Corresponds to variant dbSNP:rs60805548Ensembl. | 1 | |
Natural variantiVAR_049649 | 751 | T → A. Corresponds to variant dbSNP:rs9883888EnsemblClinVar. | 1 | |
Natural variantiVAR_049650 | 825 | R → Q. Corresponds to variant dbSNP:rs2710330Ensembl. | 1 | |
Natural variantiVAR_049651 | 858 | A → V. Corresponds to variant dbSNP:rs2710329Ensembl. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_029842 | 1 – 21 | MAFAW…LAASG → MVALSHLGSALQLGSLC in isoform 2. 2 PublicationsAdd BLAST | 21 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | X67055 mRNA Translation: CAA47439.1 Frameshift. X99854 Genomic DNA Translation: CAC79611.1 AK222757 mRNA Translation: BAD96477.1 AK223483 mRNA Translation: BAD97203.1 AC006254 Genomic DNA No translation available. BC107604 mRNA Translation: AAI07605.1 BC107605 mRNA Translation: AAI07606.1 BC107814 mRNA Translation: AAI07815.1 X14690 mRNA Translation: CAA32821.1 Frameshift. |
CCDSi | CCDS46845.1 [Q06033-1] |
PIRi | S30350 |
RefSeqi | NP_002208.3, NM_002217.3 [Q06033-1] |
Genome annotation databases
Ensembli | ENST00000449956; ENSP00000415769; ENSG00000162267 |
GeneIDi | 3699 |
KEGGi | hsa:3699 |
MANE-Selecti | ENST00000449956.3; ENSP00000415769.2; NM_002217.4; NP_002208.3 |
UCSCi | uc003dfv.3, human [Q06033-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | X67055 mRNA Translation: CAA47439.1 Frameshift. X99854 Genomic DNA Translation: CAC79611.1 AK222757 mRNA Translation: BAD96477.1 AK223483 mRNA Translation: BAD97203.1 AC006254 Genomic DNA No translation available. BC107604 mRNA Translation: AAI07605.1 BC107605 mRNA Translation: AAI07606.1 BC107814 mRNA Translation: AAI07815.1 X14690 mRNA Translation: CAA32821.1 Frameshift. |
CCDSi | CCDS46845.1 [Q06033-1] |
PIRi | S30350 |
RefSeqi | NP_002208.3, NM_002217.3 [Q06033-1] |
3D structure databases
SMRi | Q06033 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 109905, 17 interactors |
IntActi | Q06033, 7 interactors |
MINTi | Q06033 |
STRINGi | 9606.ENSP00000415769 |
Chemistry databases
DrugBanki | DB01593, Zinc DB14487, Zinc acetate DB14533, Zinc chloride DB14548, Zinc sulfate, unspecified form |
PTM databases
CarbonylDBi | Q06033 |
GlyConnecti | 1421, 10 N-Linked glycans (1 site) |
GlyGeni | Q06033, 2 sites, 10 N-linked glycans (1 site) |
iPTMneti | Q06033 |
PhosphoSitePlusi | Q06033 |
Genetic variation databases
BioMutai | ITIH3 |
DMDMi | 166203665 |
Proteomic databases
jPOSTi | Q06033 |
MassIVEi | Q06033 |
MaxQBi | Q06033 |
PaxDbi | Q06033 |
PeptideAtlasi | Q06033 |
PRIDEi | Q06033 |
ProteomicsDBi | 58409 [Q06033-1] 58410 [Q06033-2] |
Protocols and materials databases
Antibodypediai | 7471, 107 antibodies from 23 providers |
DNASUi | 3699 |
Genome annotation databases
Ensembli | ENST00000449956; ENSP00000415769; ENSG00000162267 |
GeneIDi | 3699 |
KEGGi | hsa:3699 |
MANE-Selecti | ENST00000449956.3; ENSP00000415769.2; NM_002217.4; NP_002208.3 |
UCSCi | uc003dfv.3, human [Q06033-1] |
Organism-specific databases
CTDi | 3699 |
DisGeNETi | 3699 |
GeneCardsi | ITIH3 |
HGNCi | HGNC:6168, ITIH3 |
HPAi | ENSG00000162267, Tissue enriched (liver) |
MIMi | 146650, gene |
neXtProti | NX_Q06033 |
OpenTargetsi | ENSG00000162267 |
PharmGKBi | PA29966 |
VEuPathDBi | HostDB:ENSG00000162267 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | ENOG502QPS2, Eukaryota |
GeneTreei | ENSGT00940000154554 |
InParanoidi | Q06033 |
OMAi | EFIYWIN |
OrthoDBi | 955432at2759 |
PhylomeDBi | Q06033 |
TreeFami | TF328982 |
Enzyme and pathway databases
PathwayCommonsi | Q06033 |
Reactomei | R-HSA-114608, Platelet degranulation |
SignaLinki | Q06033 |
Miscellaneous databases
BioGRID-ORCSi | 3699, 10 hits in 1033 CRISPR screens |
ChiTaRSi | ITIH3, human |
GeneWikii | ITIH3 |
GenomeRNAii | 3699 |
Pharosi | Q06033, Tbio |
PROi | PR:Q06033 |
RNActi | Q06033, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000162267, Expressed in right lobe of liver and 131 other tissues |
ExpressionAtlasi | Q06033, baseline and differential |
Genevisiblei | Q06033, 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 | ITIH3_HUMAN | |
Accessioni | Q06033Primary (citable) accession number: Q06033 Secondary accession number(s): Q3B7H5 Q99085 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | July 15, 1998 |
Last sequence update: | December 4, 2007 | |
Last modified: | February 23, 2022 | |
This is version 163 of the entry and version 2 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
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 - SIMILARITY comments
Index of protein domains and families