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UniProtKB - P19827 (ITIH1_HUMAN)

Protein

Inter-alpha-trypsin inhibitor heavy chain H1

Gene

ITIH1

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.
Contains a potential peptide which could stimulate a broad spectrum of phagocytotic cells.

GO - Molecular functioni

  • calcium ion binding 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

Names & Taxonomyi

Protein namesi
Recommended name:
Inter-alpha-trypsin inhibitor heavy chain H1
Short 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
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:ENSG00000055957.10
HGNCiHGNC:6166 ITIH1
MIMi147270 gene
neXtProtiNX_P19827

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

DisGeNETi3697
OpenTargetsiENSG00000055957
PharmGKBiPA29964

Polymorphism and mutation databases

BioMutaiITIH1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 27Sequence analysisAdd BLAST27
PropeptideiPRO_000001650628 – 341 Publication7
ChainiPRO_000001650735 – 672Inter-alpha-trypsin inhibitor heavy chain H1Add BLAST638
PropeptideiPRO_0000016508673 – 911Add BLAST239

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi60S-linked (Hex...) cysteine1 Publication1
Modified residuei129PhosphoserineCombined sources1
Disulfide bondi244 ↔ 2471 Publication
Disulfide bondi268 ↔ 5401 Publication
GlycosylationiCAR_000138285N-linked (GlcNAc...) (complex) asparagine4 Publications1
Modified residuei402PhosphothreonineCombined sources1
Modified residuei407PhosphothreonineCombined sources1
GlycosylationiCAR_000139588N-linked (GlcNAc...) (complex) asparagine3 Publications1
GlycosylationiCAR_000213653O-linked (GalNAc...) threonine1 Publication1
Modified residuei672Aspartate 1-(chondroitin 4-sulfate)-ester1
Glycosylationi750N-linked (GlcNAc...) asparagine1 Publication1

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.
The S-linked glycan is composed of two 6-carbon sugars, possibly Glc or Gal.6 Publications

Keywords - PTMi

Disulfide bond, Glycoprotein, Phosphoprotein, Proteoglycan

Proteomic databases

EPDiP19827
PaxDbiP19827
PeptideAtlasiP19827
PRIDEiP19827
ProteomicsDBi53691

PTM databases

GlyConnecti284
iPTMnetiP19827
PhosphoSitePlusiP19827
UniCarbKBiP19827

Expressioni

Gene expression databases

BgeeiENSG00000055957 Expressed in 83 organ(s), highest expression level in right lobe of liver
CleanExiHS_ITIH1
ExpressionAtlasiP19827 baseline and differential
GenevisibleiP19827 HS

Organism-specific databases

HPAiHPA041639
HPA042049

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

BioGridi109903, 5 interactors
IntActiP19827, 4 interactors
STRINGi9606.ENSP00000273283

Structurei

3D structure databases

ProteinModelPortaliP19827
SMRiP19827
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini37 – 166VITPROSITE-ProRule annotationAdd BLAST130
Domaini290 – 450VWFAPROSITE-ProRule annotationAdd BLAST161

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni387 – 911Hyaluronan-bindingAdd BLAST525

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi181 – 184Phagocytosis uptake signalSequence analysis4

Sequence similaritiesi

Belongs to the ITIH family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiENOG410IEJB Eukaryota
COG2304 LUCA
GeneTreeiENSGT00550000074468
HOGENOMiHOG000000680
HOVERGENiHBG057734
InParanoidiP19827
KOiK19014
OMAiMSMENNG
OrthoDBiEOG091G01HF
PhylomeDBiP19827
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 (3+)i

Sequence statusi: Complete.

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

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 3 described isoforms and 4 potential isoforms that are computationally mapped.iShow all

Isoform 1 (identifier: P19827-1) [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
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
Length:911
Mass (Da):101,389
Last modified:July 15, 1998 - v3
Checksum:i8FE715FF223FC917
GO
Isoform 2 (identifier: P19827-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-142: Missing.

Note: No experimental confirmation available.
Show »
Length:769
Mass (Da):86,192
Checksum:i9447D42CB083BC9F
GO
Isoform 3 (identifier: P19827-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-288: Missing.

Note: No experimental confirmation available.
Show »
Length:623
Mass (Da):69,495
Checksum:iEBB1BB3250D74DA7
GO

Computationally mapped potential isoform sequencesi

There are 4 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
F8WAS2F8WAS2_HUMAN
Inter-alpha-trypsin inhibitor heavy...
ITIH1
213Annotation score:
B5MCP1B5MCP1_HUMAN
Inter-alpha-trypsin inhibitor heavy...
ITIH1
277Annotation score:
H7C0N0H7C0N0_HUMAN
Inter-alpha-trypsin inhibitor heavy...
ITIH1
464Annotation score:
H7C5I0H7C5I0_HUMAN
Inter-alpha-trypsin inhibitor heavy...
ITIH1
74Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti51V → T AA sequence (PubMed:2476436).Curated1
Sequence conflicti54R → A AA sequence (PubMed:2476436).Curated1
Sequence conflicti266K → E in CAA45188 (PubMed:1380832).Curated1
Sequence conflicti539T → A in BAH12794 (PubMed:14702039).Curated1
Sequence conflicti798V → A in BAH12794 (PubMed:14702039).Curated1

Mass spectrometryi

Molecular mass is 76258 Da from positions 28 - 672. Determined by MALDI. 1 Publication

Polymorphismi

There are 3 common alleles; ITIH1*1 with Glu-585/Gln-595, ITIH1*2 with Val-585/Arg-595 and ITIH1*3 with Glu-585/Arg-595.

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_011873263S → T1 PublicationCorresponds to variant dbSNP:rs1042777Ensembl.1
Natural variantiVAR_004019585E → V in allele ITIH1*2. 3 PublicationsCorresponds to variant dbSNP:rs678EnsemblClinVar.1
Natural variantiVAR_004020595Q → R in allele ITIH1*2 and allele ITIH1*3. 3 PublicationsCorresponds to variant dbSNP:rs1042779EnsemblClinVar.1
Natural variantiVAR_011874695G → C. Corresponds to variant dbSNP:rs1042904Ensembl.1
Natural variantiVAR_011875844D → E. Corresponds to variant dbSNP:rs1042849Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0454191 – 288Missing in isoform 3. 1 PublicationAdd BLAST288
Alternative sequenceiVSP_0454201 – 142Missing in isoform 2. 1 PublicationAdd BLAST142

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
CCDSiCCDS2864.1 [P19827-1]
CCDS54595.1 [P19827-3]
PIRiS39527 A39967
RefSeqiNP_001159906.1, NM_001166434.2 [P19827-2]
NP_001159907.1, NM_001166435.2 [P19827-3]
NP_002206.2, NM_002215.3 [P19827-1]
UniGeneiHs.420257
Hs.76716

Genome annotation databases

EnsembliENST00000273283; ENSP00000273283; ENSG00000055957 [P19827-1]
ENST00000537050; ENSP00000443847; ENSG00000055957 [P19827-3]
GeneIDi3697
KEGGihsa:3697
UCSCiuc003dfs.4 human [P19827-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar 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
CCDSiCCDS2864.1 [P19827-1]
CCDS54595.1 [P19827-3]
PIRiS39527 A39967
RefSeqiNP_001159906.1, NM_001166434.2 [P19827-2]
NP_001159907.1, NM_001166435.2 [P19827-3]
NP_002206.2, NM_002215.3 [P19827-1]
UniGeneiHs.420257
Hs.76716

3D structure databases

ProteinModelPortaliP19827
SMRiP19827
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi109903, 5 interactors
IntActiP19827, 4 interactors
STRINGi9606.ENSP00000273283

PTM databases

GlyConnecti284
iPTMnetiP19827
PhosphoSitePlusiP19827
UniCarbKBiP19827

Polymorphism and mutation databases

BioMutaiITIH1

Proteomic databases

EPDiP19827
PaxDbiP19827
PeptideAtlasiP19827
PRIDEiP19827
ProteomicsDBi53691

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000273283; ENSP00000273283; ENSG00000055957 [P19827-1]
ENST00000537050; ENSP00000443847; ENSG00000055957 [P19827-3]
GeneIDi3697
KEGGihsa:3697
UCSCiuc003dfs.4 human [P19827-1]

Organism-specific databases

CTDi3697
DisGeNETi3697
EuPathDBiHostDB:ENSG00000055957.10
GeneCardsiITIH1
HGNCiHGNC:6166 ITIH1
HPAiHPA041639
HPA042049
MIMi147270 gene
neXtProtiNX_P19827
OpenTargetsiENSG00000055957
PharmGKBiPA29964
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IEJB Eukaryota
COG2304 LUCA
GeneTreeiENSGT00550000074468
HOGENOMiHOG000000680
HOVERGENiHBG057734
InParanoidiP19827
KOiK19014
OMAiMSMENNG
OrthoDBiEOG091G01HF
PhylomeDBiP19827
TreeFamiTF328982

Miscellaneous databases

ChiTaRSiITIH1 human
GeneWikiiITIH1
GenomeRNAii3697
PROiPR:P19827
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000055957 Expressed in 83 organ(s), highest expression level in right lobe of liver
CleanExiHS_ITIH1
ExpressionAtlasiP19827 baseline and differential
GenevisibleiP19827 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 nameiITIH1_HUMAN
AccessioniPrimary (citable) accession number: P19827
Secondary accession number(s): A8K9N5
, B2RAH9, B7Z558, B7Z8C0, F5H165, F5H7Y8, P78455, Q01746, Q562G1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 1, 1991
Last sequence update: July 15, 1998
Last modified: September 12, 2018
This is version 179 of the entry and version 3 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
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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