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UniProtKB - Q06033 (ITIH3_HUMAN)

Protein

Inter-alpha-trypsin inhibitor heavy chain H3

Gene

ITIH3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

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
View the complete GO annotation on QuickGO ...

GO - Biological processi

View the complete GO annotation on QuickGO ...

Keywordsi

Molecular functionProtease inhibitor, Serine protease inhibitor

Enzyme and pathway databases

ReactomeiR-HSA-114608 Platelet degranulation

Names & Taxonomyi

Protein namesi
Recommended name:
Inter-alpha-trypsin inhibitor heavy chain H3
Short 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
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

EuPathDBiHostDB:ENSG00000162267.12
HGNCiHGNC:6168 ITIH3
MIMi146650 gene
neXtProtiNX_Q06033

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Secreted

Pathology & Biotechi

Organism-specific databases

DisGeNETi3699
OpenTargetsiENSG00000162267
PharmGKBiPA29966

Polymorphism and mutation databases

BioMutaiITIH3
DMDMi166203665

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 20Sequence analysisAdd BLAST20
PropeptideiPRO_000001652921 – 34Add BLAST14
ChainiPRO_000001653035 – 651Inter-alpha-trypsin inhibitor heavy chain H3Add BLAST617
PropeptideiPRO_0000016531652 – 890Add BLAST239

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi91N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi580N-linked (GlcNAc...) asparagine1 Publication1
Modified residuei651Aspartate 1-(chondroitin 4-sulfate)-ester1

Post-translational modificationi

Heavy chains are linked to bikunin via chondroitin 4-sulfate esterified to the alpha-carboxyl of the C-terminal aspartate after propeptide cleavage.

Keywords - PTMi

Glycoprotein, Proteoglycan

Proteomic databases

MaxQBiQ06033
PaxDbiQ06033
PeptideAtlasiQ06033
PRIDEiQ06033
ProteomicsDBi58409
58410 [Q06033-2]

PTM databases

CarbonylDBiQ06033
GlyConnecti1421
iPTMnetiQ06033
PhosphoSitePlusiQ06033

Expressioni

Gene expression databases

BgeeiENSG00000162267 Expressed in 111 organ(s), highest expression level in right lobe of liver
CleanExiHS_ITIH3
ExpressionAtlasiQ06033 baseline and differential
GenevisibleiQ06033 HS

Organism-specific databases

HPAiHPA017373

Interactioni

Subunit structurei

I-alpha-I plasma protease inhibitors are assembled from one or two heavy chains (H1, H2 or H3) and one light chain, bikunin. Inter-alpha-inhibitor (I-alpha-I) is composed of H1, H2 and bikunin, inter-alpha-like inhibitor (I-alpha-LI) of H2 and bikunin, and pre-alpha-inhibitor (P-alpha-I) of H3 and bikunin.

Protein-protein interaction databases

BioGridi109905, 8 interactors
IntActiQ06033, 4 interactors
MINTiQ06033
STRINGi9606.ENSP00000415769

Structurei

3D structure databases

ProteinModelPortaliQ06033
SMRiQ06033
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini29 – 158VITPROSITE-ProRule annotationAdd BLAST130
Domaini284 – 467VWFAPROSITE-ProRule annotationAdd BLAST184

Sequence similaritiesi

Belongs to the ITIH family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiENOG410IEJB Eukaryota
COG2304 LUCA
GeneTreeiENSGT00550000074468
HOGENOMiHOG000000680
HOVERGENiHBG057734
InParanoidiQ06033
OMAiVVDSHRM
OrthoDBiEOG091G01HF
PhylomeDBiQ06033
TreeFamiTF328982

Family and domain databases

Gene3Di3.40.50.410, 1 hit
InterProiView protein in InterPro
IPR010600 ITI_HC_C
IPR013694 VIT
IPR002035 VWF_A
IPR036465 vWFA_dom_sf
PfamiView protein in Pfam
PF06668 ITI_HC_C, 1 hit
PF08487 VIT, 1 hit
PF00092 VWA, 1 hit
SMARTiView protein in SMART
SM00609 VIT, 1 hit
SM00327 VWA, 1 hit
SUPFAMiSSF53300 SSF53300, 1 hit
PROSITEiView protein in PROSITE
PS51468 VIT, 1 hit
PS50234 VWFA, 1 hit

Sequences (2+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: Q06033-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        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
Length:890
Mass (Da):99,849
Last modified:December 4, 2007 - v2
Checksum:i03B2B768784CB440
GO
Isoform 2 (identifier: Q06033-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-21: MAFAWWPCLILALLSSLAASG → MVALSHLGSALQLGSLC

Show »
Length:886
Mass (Da):99,328
Checksum:i0B717C8073BE7EF6
GO

Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
E7ET33E7ET33_HUMAN
Inter-alpha-trypsin inhibitor heavy...
ITIH3
698Annotation score:
A0A087WW43A0A087WW43_HUMAN
Inter-alpha-trypsin inhibitor heavy...
ITIH3
670Annotation score:

Sequence cautioni

The sequence CAA32821 differs from that shown. Reason: Frameshift at positions 732, 736 and 739.Curated
The sequence CAA47439 differs from that shown. Reason: Frameshift at positions 21, 732, 736 and 739.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti113K → N in BAD97203 (Ref. 3) Curated1
Sequence conflicti113K → N in BAD96477 (Ref. 3) Curated1
Sequence conflicti348R → K in CAA32821 (PubMed:2465147).Curated1
Sequence conflicti361N → G in CAA32821 (PubMed:2465147).Curated1
Sequence conflicti515F → L in CAC79611 (PubMed:9756925).Curated1
Sequence conflicti667F → L in BAD97203 (Ref. 3) Curated1
Sequence conflicti667F → L in BAD96477 (Ref. 3) Curated1
Sequence conflicti716A → R in CAA47439 (PubMed:7681778).Curated1
Sequence conflicti716A → R in CAA32821 (PubMed:2465147).Curated1
Sequence conflicti851Q → H in CAA32821 (PubMed:2465147).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_049647315Q → K1 PublicationCorresponds to variant dbSNP:rs3617Ensembl.1
Natural variantiVAR_049648340T → M. Corresponds to variant dbSNP:rs35271262Ensembl.1
Natural variantiVAR_061275640P → L. Corresponds to variant dbSNP:rs60805548Ensembl.1
Natural variantiVAR_049649751T → A. Corresponds to variant dbSNP:rs9883888Ensembl.1
Natural variantiVAR_049650825R → Q. Corresponds to variant dbSNP:rs2710330Ensembl.1
Natural variantiVAR_049651858A → V. Corresponds to variant dbSNP:rs2710329Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0298421 – 21MAFAW…LAASG → MVALSHLGSALQLGSLC in isoform 2. 2 PublicationsAdd BLAST21

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.
CCDSiCCDS46845.1 [Q06033-1]
PIRiS30350
RefSeqiNP_002208.3, NM_002217.3 [Q06033-1]
UniGeneiHs.76716

Genome annotation databases

EnsembliENST00000449956; ENSP00000415769; ENSG00000162267 [Q06033-1]
GeneIDi3699
KEGGihsa:3699
UCSCiuc003dfv.3 human [Q06033-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar 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.
CCDSiCCDS46845.1 [Q06033-1]
PIRiS30350
RefSeqiNP_002208.3, NM_002217.3 [Q06033-1]
UniGeneiHs.76716

3D structure databases

ProteinModelPortaliQ06033
SMRiQ06033
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi109905, 8 interactors
IntActiQ06033, 4 interactors
MINTiQ06033
STRINGi9606.ENSP00000415769

PTM databases

CarbonylDBiQ06033
GlyConnecti1421
iPTMnetiQ06033
PhosphoSitePlusiQ06033

Polymorphism and mutation databases

BioMutaiITIH3
DMDMi166203665

Proteomic databases

MaxQBiQ06033
PaxDbiQ06033
PeptideAtlasiQ06033
PRIDEiQ06033
ProteomicsDBi58409
58410 [Q06033-2]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000449956; ENSP00000415769; ENSG00000162267 [Q06033-1]
GeneIDi3699
KEGGihsa:3699
UCSCiuc003dfv.3 human [Q06033-1]

Organism-specific databases

CTDi3699
DisGeNETi3699
EuPathDBiHostDB:ENSG00000162267.12
GeneCardsiITIH3
H-InvDBiHIX0024346
HGNCiHGNC:6168 ITIH3
HPAiHPA017373
MIMi146650 gene
neXtProtiNX_Q06033
OpenTargetsiENSG00000162267
PharmGKBiPA29966
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IEJB Eukaryota
COG2304 LUCA
GeneTreeiENSGT00550000074468
HOGENOMiHOG000000680
HOVERGENiHBG057734
InParanoidiQ06033
OMAiVVDSHRM
OrthoDBiEOG091G01HF
PhylomeDBiQ06033
TreeFamiTF328982

Enzyme and pathway databases

ReactomeiR-HSA-114608 Platelet degranulation

Miscellaneous databases

ChiTaRSiITIH3 human
GeneWikiiITIH3
GenomeRNAii3699
PROiPR:Q06033
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000162267 Expressed in 111 organ(s), highest expression level in right lobe of liver
CleanExiHS_ITIH3
ExpressionAtlasiQ06033 baseline and differential
GenevisibleiQ06033 HS

Family and domain databases

Gene3Di3.40.50.410, 1 hit
InterProiView protein in InterPro
IPR010600 ITI_HC_C
IPR013694 VIT
IPR002035 VWF_A
IPR036465 vWFA_dom_sf
PfamiView protein in Pfam
PF06668 ITI_HC_C, 1 hit
PF08487 VIT, 1 hit
PF00092 VWA, 1 hit
SMARTiView protein in SMART
SM00609 VIT, 1 hit
SM00327 VWA, 1 hit
SUPFAMiSSF53300 SSF53300, 1 hit
PROSITEiView protein in PROSITE
PS51468 VIT, 1 hit
PS50234 VWFA, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiITIH3_HUMAN
AccessioniPrimary (citable) accession number: Q06033
Secondary accession number(s): Q3B7H5
, Q53F06, Q6LAM2, Q99085
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: December 4, 2007
Last modified: October 10, 2018
This is version 144 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. SIMILARITY comments
    Index of protein domains and families
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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