Itih1 inter-alpha trypsin inhibitor, heavy chain 1 [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Itih1provided by MGI
Official Full Name: inter-alpha trypsin inhibitor, heavy chain 1provided by MGI
Primary source: MGI:MGI:96618
See related: Ensembl:ENSMUSG00000006529 AllianceGenome:MGI:96618
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: Intin1; Itih-1; ITI-HC1
Summary: This gene encodes a heavy chain of inter-alpha trypsin inhibitor (IaI) family of plasma serine protease inhibitors. IaI proteins are protein-glycosaminoglycan-protein complexes comprised of two heavy chains and a light chain. The encoded protein covalently associates with the light chain via a chondroitin sulfate moiety. Intravenous administration of the encoded protein improved survival of mice after infection with Escherichia coli. This gene is located adjacent to two other IaI heavy chain genes. Alternative splicing results in multiple transcript variants encoding different isoforms that may undergo similar proteolytic processing to generate mature protein. [provided by RefSeq, Oct 2015]
Expression: Biased expression in liver adult (RPKM 180.0), liver E18 (RPKM 77.5) and 2 other tissues See more
Orthologs: human all
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Genomic context

Location:: 14 B; 14 19.09 cM

Exon count:: 22

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	14	NC_000080.7 (30651137..30665246, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	14	NC_000080.6 (30929180..30943289, complement)

Chromosome 14 - NC_000080.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

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Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

PTX3 directly interacts with inter-alpha-trypsin inhibitor and has a role in hyaluronan organization and cumulus oophorus expansion
Title: PTX3 interacts with inter-alpha-trypsin inhibitor: implications for hyaluronan organization and cumulus oophorus expansion.
In a sepsis model inter-alpha-trypsin inhibitor inhibits complement activation through the classical and alternative pathways, inhibits complement-dependent phagocytosis in vitro, and reduces complement-dependent lung injury in vivo.
Title: Inter-alpha-trypsin inhibitor attenuates complement activation and complement-induced lung injury.

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Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (2)
Targeted (2)

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables carbohydrate binding	ISO Inferred from Sequence Orthology more info
enables hyaluronic acid binding	ISO Inferred from Sequence Orthology more info
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables serine-type endopeptidase inhibitor activity	IEA Inferred from Electronic Annotation more info

Process	Evidence Code	Pubs
acts_upstream_of_or_within extracellular matrix organization	IDA Inferred from Direct Assay more info	PubMed
involved_in hyaluronan metabolic process	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
located_in collagen-containing extracellular matrix	HDA more info	PubMed
located_in extracellular matrix	IDA Inferred from Direct Assay more info	PubMed
located_in extracellular region	IEA Inferred from Electronic Annotation more info

General protein information

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Preferred Names: inter-alpha-trypsin inhibitor heavy chain H1

Names: inter-alpha (globulin) inhibitor, H1 polypeptide

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_001306078.1 → NP_001293007.1 inter-alpha-trypsin inhibitor heavy chain H1 isoform 2 precursor

Status: REVIEWED

Description

Transcript Variant: This variant (2) uses an alternate in-frame splice site in the 5' coding region compared to variant 1. This variant encodes isoform 2, which is longer than isoform 1. This isoform (2) may undergo proteolytic processing similar to isoform 1.

Source sequence(s)

AK149500, CT025528

Consensus CDS

CCDS79287.1

UniProtKB/TrEMBL

F8WJ05, Q8K159

Related

ENSMUSP00000006704.8, ENSMUST00000006704.17

Conserved Domains (5) summary

smart00609
Location:34 → 167

VIT; Vault protein Inter-alpha-Trypsin domain

cd01461
Location:290 → 471

vWA_interalpha_trypsin_inhibitor; vWA_interalpha trypsin inhibitor (ITI): ITI is a glycoprotein composed of three polypeptides- two heavy chains and one light chain (bikunin). Bikunin confers the protease-inhibitor function while the heavy chains are involved in rendering stability to ...

TIGR03788
Location:59 → 610

marine_srt_targ; marine proteobacterial sortase target protein

pfam06668
Location:706 → 891

ITI_HC_C; Inter-alpha-trypsin inhibitor heavy chain C-terminus

cl19107
Location:526 → 620

SPFH_like; core domain of the SPFH (stomatin, prohibitin, flotillin, and HflK/C) superfamily
NM_008406.3 → NP_032432.2 inter-alpha-trypsin inhibitor heavy chain H1 isoform 1 preproprotein

See identical proteins and their annotated locations for NP_032432.2

Status: REVIEWED

Description

Transcript Variant: This variant (1) represents the shorter transcript and encodes the shorter isoform (1).

Source sequence(s)

AK149500

Consensus CDS

CCDS49435.1

UniProtKB/Swiss-Prot

Q3UEI9, Q61702

UniProtKB/TrEMBL

Q8K159

Related

ENSMUSP00000126449.2, ENSMUST00000163118.2

Conserved Domains (5) summary

smart00609
Location:34 → 163

VIT; Vault protein Inter-alpha-Trypsin domain

cd01461
Location:286 → 467

vWA_interalpha_trypsin_inhibitor; vWA_interalpha trypsin inhibitor (ITI): ITI is a glycoprotein composed of three polypeptides- two heavy chains and one light chain (bikunin). Bikunin confers the protease-inhibitor function while the heavy chains are involved in rendering stability to ...

TIGR03788
Location:55 → 606

marine_srt_targ; marine proteobacterial sortase target protein

pfam06668
Location:702 → 887

ITI_HC_C; Inter-alpha-trypsin inhibitor heavy chain C-terminus

cl19107
Location:522 → 616

SPFH_like; core domain of the SPFH (stomatin, prohibitin, flotillin, and HflK/C) superfamily

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

NC_000080.7 Reference GRCm39 C57BL/6J

Range

30651137..30665246 complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_006518584.3 → XP_006518647.1 inter-alpha-trypsin inhibitor heavy chain H1 isoform X1

UniProtKB/TrEMBL

Q91WU7

Conserved Domains (3) summary

cd01461
Location:147 → 328

vWA_interalpha_trypsin_inhibitor; vWA_interalpha trypsin inhibitor (ITI): ITI is a glycoprotein composed of three polypeptides- two heavy chains and one light chain (bikunin). Bikunin confers the protease-inhibitor function while the heavy chains are involved in rendering stability to ...

pfam06668
Location:562 → 748

ITI_HC_C; Inter-alpha-trypsin inhibitor heavy chain C-terminus

cl02699
Location:1 → 24

VIT; Vault protein inter-alpha-trypsin domain