Free for academic non-profit institutions. Other users need a Commercial license

Aliases for ITIH3 Gene

Aliases for ITIH3 Gene

  • Inter-Alpha-Trypsin Inhibitor Heavy Chain 3 2 3
  • Inter-Alpha (Globulin) Inhibitor, H3 Polypeptide 2 3
  • Inter-Alpha-Trypsin Inhibitor Heavy Chain H3 2 3
  • Serum-Derived Hyaluronan-Associated Protein 3 4
  • Inter-Alpha (Globulin) Inhibitor H3 2 3
  • Inter-Alpha-Inhibitor Heavy Chain 3 3 4
  • ITI Heavy Chain H3 3 4
  • ITI-HC3 3 4
  • SHAP 3 4
  • Pre-Alpha (Globulin) Inhibitor, H3 Polypeptide 3
  • Pre-Alpha (Globulin) Inhibitor 2
  • H3 Polypeptide 2
  • H3P 3

External Ids for ITIH3 Gene

Previous GeneCards Identifiers for ITIH3 Gene

  • GC03P051920
  • GC03P052080
  • GC03P052683
  • GC03P052786
  • GC03P052803

Summaries for ITIH3 Gene

Entrez Gene Summary for ITIH3 Gene

  • The inter-alpha-trypsin inhibitors (ITI) are a family of structurally related plasma serine protease inhibitors involved in extracellular matrix stabilization and in prevention of tumor metastasis. The ITI family contains multiple proteins made up of a light chain (see MIM 176870) and a variable number of heavy chains (Salier et al., 1987 [PubMed 2446322]; Himmelfarb et al., 2004 [PubMed 14744536]).[supplied by OMIM, Nov 2009]

GeneCards Summary for ITIH3 Gene

ITIH3 (Inter-Alpha-Trypsin Inhibitor Heavy Chain 3) is a Protein Coding gene. Among its related pathways are Cell adhesion_Cell-matrix glycoconjugates. GO annotations related to this gene include serine-type endopeptidase inhibitor activity and endopeptidase inhibitor activity. An important paralog of this gene is ITIH1.

UniProtKB/Swiss-Prot for ITIH3 Gene

  • 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

Gene Wiki entry for ITIH3 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for ITIH3 Gene

Genomics for ITIH3 Gene

Regulatory Elements for ITIH3 Gene

Genomic Location for ITIH3 Gene

52,794,768 bp from pter
52,809,011 bp from pter
14,244 bases
Plus strand

Genomic View for ITIH3 Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for ITIH3 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for ITIH3 Gene

Proteins for ITIH3 Gene

  • Protein details for ITIH3 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Inter-alpha-trypsin inhibitor heavy chain H3
    Protein Accession:
    Secondary Accessions:
    • Q3B7H5
    • Q53F06
    • Q6LAM2
    • Q99085

    Protein attributes for ITIH3 Gene

    890 amino acids
    Molecular mass:
    99849 Da
    Quaternary structure:
    • 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
    • Sequence=CAA32821.1; Type=Frameshift; Positions=732, 736, 739; Evidence={ECO:0000305}; Sequence=CAA47439.1; Type=Frameshift; Positions=21, 732, 736, 739; Evidence={ECO:0000305};

    Alternative splice isoforms for ITIH3 Gene


neXtProt entry for ITIH3 Gene

Proteomics data for ITIH3 Gene at MOPED

Post-translational modifications for ITIH3 Gene

  • Heavy chains are linked to bikunin via chondroitin 4-sulfate esterified to the alpha-carboxyl of the C-terminal aspartate after propeptide cleavage
  • Glycosylation at Asn91 and Asn580
  • Modification sites at PhosphoSitePlus

Other Protein References for ITIH3 Gene

ENSEMBL proteins:
REFSEQ proteins:

No data available for DME Specific Peptides for ITIH3 Gene

Domains for ITIH3 Gene

Suggested Antigen Peptide Sequences for ITIH3 Gene

GenScript: Design optimal peptide antigens:

Graphical View of Domain Structure for InterPro Entry



  • Q06033
  • Contains 1 VIT domain.
  • Contains 1 VWFA domain.
  • Belongs to the ITIH family.
genes like me logo Genes that share domains with ITIH3: view

No data available for Gene Families for ITIH3 Gene

Function for ITIH3 Gene

Molecular function for ITIH3 Gene

GENATLAS Biochemistry: trypsin inhibitor,inter-alpha- (protein HC),heavy chain 3
UniProtKB/Swiss-Prot Function: 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

Gene Ontology (GO) - Molecular Function for ITIH3 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004866 endopeptidase inhibitor activity TAS 2465147
GO:0004867 serine-type endopeptidase inhibitor activity IEA --
genes like me logo Genes that share ontologies with ITIH3: view

Animal Model Products

CRISPR Products

No data available for Enzyme Numbers (IUBMB) , Phenotypes , Animal Models , miRNA , Transcription Factor Targeting and HOMER Transcription for ITIH3 Gene

Localization for ITIH3 Gene

Subcellular locations from UniProtKB/Swiss-Prot for ITIH3 Gene


Subcellular locations from

Jensen Localization Image for ITIH3 Gene COMPARTMENTS Subcellular localization image for ITIH3 gene
Compartment Confidence
extracellular 5
endoplasmic reticulum 2
golgi apparatus 1
plasma membrane 1

Gene Ontology (GO) - Cellular Components for ITIH3 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005576 extracellular region NAS 14718574
GO:0070062 extracellular exosome IDA 23533145
genes like me logo Genes that share ontologies with ITIH3: view

Pathways for ITIH3 Gene

genes like me logo Genes that share pathways with ITIH3: view

Pathways by source for ITIH3 Gene

1 GeneGo (Thomson Reuters) pathway for ITIH3 Gene

Interacting Proteins for ITIH3 Gene

STRING Interaction Network Preview (showing 2 interactants - click image to see details)
Selected Interacting proteins: ENSP00000415769 Q06033-ITIH3_HUMAN for ITIH3 Gene via STRING I2D

Gene Ontology (GO) - Biological Process for ITIH3 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0010951 negative regulation of endopeptidase activity TAS 2465147
GO:0030212 hyaluronan metabolic process IEA --
genes like me logo Genes that share ontologies with ITIH3: view

Transcripts for ITIH3 Gene

Unigene Clusters for ITIH3 Gene

Inter-alpha-trypsin inhibitor heavy chain 3:
Representative Sequences:

CRISPR Products

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for ITIH3

Primer Products

  • OriGene qSTAR qPCR primer pairs in human,mouse,rat for ITIH3

Flow Cytometry Products

Alternative Splicing Database (ASD) splice patterns (SP) for ITIH3 Gene

ExUns: 1 ^ 2 ^ 3 ^ 4a · 4b ^ 5a · 5b · 5c ^ 6a · 6b ^ 7 ^ 8 ^ 9a · 9b ^ 10a · 10b · 10c ^ 11a · 11b ^ 12 ^ 13 ^ 14a · 14b · 14c ^ 15 ^ 16 ^
SP1: - -
SP3: -
SP4: -
SP5: -
SP8: - -

ExUns: 17 ^ 18 ^ 19 ^ 20 ^ 21 ^ 22

Relevant External Links for ITIH3 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for ITIH3 Gene

mRNA expression in normal human tissues for ITIH3 Gene

mRNA differential expression in normal tissues according to GTEx for ITIH3 Gene

This gene is overexpressed in Liver (45.3).

Integrated Proteomics: protein expression from ProteomicsDB, MOPED, and MaxQB for ITIH3 Gene

SOURCE GeneReport for Unigene cluster for ITIH3 Gene Hs.76716

genes like me logo Genes that share expressions with ITIH3: view

In Situ Assay Products

No data available for mRNA expression in embryonic tissues and stem cells from LifeMap Discovery and mRNA Expression by UniProt/SwissProt for ITIH3 Gene

Orthologs for ITIH3 Gene

This gene was present in the common ancestor of chordates.

Orthologs for ITIH3 Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia ITIH3 35
  • 99.36 (n)
  • 98.65 (a)
ITIH3 36
  • 99 (a)
(Bos Taurus)
Mammalia ITIH3 35
  • 87.53 (n)
  • 86.29 (a)
ITIH3 36
  • 86 (a)
(Canis familiaris)
Mammalia ITIH3 35
  • 88.35 (n)
  • 88.54 (a)
ITIH3 36
  • 88 (a)
(Mus musculus)
Mammalia Itih3 35
  • 84.68 (n)
  • 85.14 (a)
Itih3 16
Itih3 36
  • 85 (a)
(Monodelphis domestica)
Mammalia ITIH3 36
  • 49 (a)
(Ornithorhynchus anatinus)
Mammalia -- 36
  • 70 (a)
-- 36
  • 74 (a)
-- 36
  • 57 (a)
-- 36
  • 52 (a)
(Rattus norvegicus)
Mammalia Itih3 35
  • 84.12 (n)
  • 84.2 (a)
(Gallus gallus)
Aves -- 36
  • 64 (a)
ITIH3 35
  • 66.31 (n)
  • 66.35 (a)
(Anolis carolinensis)
Reptilia -- 36
  • 60 (a)
-- 36
  • 64 (a)
African clawed frog
(Xenopus laevis)
Amphibia Xl.5040 35
rainbow trout
(Oncorhynchus mykiss)
Actinopterygii Omy.9191 35
(Danio rerio)
Actinopterygii CR558302.3 36
  • 48 (a)
LOC564761 35
  • 58.81 (n)
  • 51.9 (a)
zgc:110377 36
  • 49 (a)
Species with no ortholog for ITIH3:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African malaria mosquito (Anopheles gambiae)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • baker's yeast (Saccharomyces cerevisiae)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • fruit fly (Drosophila melanogaster)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rice (Oryza sativa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea squirt (Ciona intestinalis)
  • sea squirt (Ciona savignyi)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • thale cress (Arabidopsis thaliana)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • tropical clawed frog (Silurana tropicalis)
  • wheat (Triticum aestivum)
  • worm (Caenorhabditis elegans)

Evolution for ITIH3 Gene

Gene Tree for ITIH3 (if available)
Gene Tree for ITIH3 (if available)

Paralogs for ITIH3 Gene

Paralogs for ITIH3 Gene

Selected SIMAP similar genes for ITIH3 Gene using alignment to 2 proteins:

genes like me logo Genes that share paralogs with ITIH3: view

Variants for ITIH3 Gene

Sequence variations from dbSNP and Humsavar for ITIH3 Gene

SNP ID Clin Chr 03 pos Sequence Context AA Info Type MAF
rs3617 - 52,799,789(-) CTCTT(G/T)CATAT missense, reference
rs736408 -- 52,801,338(-) ATCCT(A/G)TTGAA intron-variant
rs740920 -- 52,802,201(-) GACTG(C/T)GTCCT intron-variant
rs1050056 -- 52,808,575(+) CAGAA(A/G)GGATG reference, missense
rs2071508 -- 52,792,830(+) ATGAC(A/G)ACTCT upstream-variant-2KB

Structural Variations from Database of Genomic Variants (DGV) for ITIH3 Gene

Variant ID Type Subtype PubMed ID
nsv834698 CNV Gain 17160897
nsv508928 CNV Insertion 20534489
nsv876797 CNV Loss 21882294
nsv3822 CNV Insertion 18451855

Relevant External Links for ITIH3 Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for ITIH3 Gene

Disorders for ITIH3 Gene

Relevant External Links for ITIH3

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with ITIH3: view

No data available for UniProtKB/Swiss-Prot for ITIH3 Gene

Publications for ITIH3 Gene

  1. Tandem orientation of the inter-alpha-trypsin inhibitor heavy chain H1 and H3 genes. (PMID: 7522574) Diarra-Mehrpour M. … Martin J.-P. (Biochim. Biophys. Acta 1994) 3 4 23
  2. Human inter-alpha-trypsin inhibitor heavy chain H3 gene. Genomic organization, promoter analysis, and gene linkage. (PMID: 9756925) Diarra-Mehrpour M. … Martin J.-P. (J. Biol. Chem. 1998) 3 4 23
  3. A functional SNP in ITIH3 is associated with susceptibility to myocardial infarction. (PMID: 17211523) Ebana Y. … Tanaka T. (J. Hum. Genet. 2007) 3 23 48
  4. Human plasma inter-alpha-trypsin inhibitor is encoded by four genes on three chromosomes. (PMID: 2465147) Diarra-Mehrpour M. … Martin J.-P. (Eur. J. Biochem. 1989) 2 3 4
  5. Analysis of inter-alpha-trypsin inhibitor and a novel trypsin inhibitor, pre-alpha-trypsin inhibitor, from human plasma. Polypeptide chain stoichiometry and assembly by glycan. (PMID: 2476436) Enghild J.J. … Salvesen G. (J. Biol. Chem. 1989) 3 4

Products for ITIH3 Gene

Sources for ITIH3 Gene

Back to Top