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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ITIH2
inter-alpha-trypsin inhibitor heavy chain 2
Chromosome 10 · 10p14
NCBI Gene: 3698Ensembl: ENSG00000151655.20HGNC: HGNC:6167UniProt: P19823
69PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
extracellular exosomeblood microparticleprotein bindinghyaluronic acid bindingacute tonsillitisneurodegenerative diseasetype 2 diabetes mellitusnon-alcoholic fatty liver disease
✦AI Summary

ITIH2 (inter-alpha-trypsin inhibitor heavy chain 2) is a serum and extracellular matrix protein that functions as a hyaluronan-binding carrier protein regulating hyaluronan localization, synthesis, and degradation 1. In cancer biology, ITIH2 is transcriptionally upregulated by the EMT-inducing transcription factor ZEB1 in mesenchymal-like lung cancer cells, and its depletion reduces hyaluronan matrix formation and cancer cell migration and invasion 1. In hepatocellular carcinoma, tumor-derived ITIH2 promotes angiogenesis by activating the PI3K/AKT signaling pathway in vascular endothelial cells through THBS1 ubiquitination-dependent regulation, without directly affecting tumor cell proliferation 2. ITIH2 demonstrates clinical significance as a disease biomarker across multiple conditions: it associates with COVID-19 severity as an early predictor 3, serves as a component of diagnostic signatures for pancreatic ductal adenocarcinoma detection 4, correlates with systemic lupus erythematosus disease activity and renal damage 5, and is more abundant in severe COVID-19 survivors compared to non-survivors 6. Additionally, ITIH2 undergoes aging-associated structural changes in cerebrospinal fluid that associate with neurodegeneration-related clinical features 7, and is present in plasma-derived extracellular vesicles of hepatocellular carcinoma patients 8. These findings establish ITIH2 as a multifunctional extracellular protein with roles in tumor progression, immune homeostasis, and disease monitoring.

Sources cited
1
ITIH2 is an HA-binding protein secreted from mesenchymal cancer cells, upregulated by ZEB1, and its depletion reduces HA matrix formation and cancer cell migration/invasion
PMID: 40178908
2
Tumor-derived ITIH2 promotes hepatocellular carcinoma angiogenesis by activating PI3K/AKT pathway via THBS1 ubiquitination in vascular endothelial cells
PMID: 41616982
3
ITIH2 is identified as a potential early predictor of COVID-19 severity among differentially expressed proteins
PMID: 36217943
4
ITIH2 is part of an eight-gene extracellular vesicle long RNA signature for pancreatic ductal adenocarcinoma detection with high diagnostic accuracy
PMID: 31562239
5
Urine ITIH2 expression correlates with systemic lupus erythematosus disease activity and renal damage
PMID: 35870194
6
ITIH2 is more abundant in severe COVID-19 survivors compared to non-survivors and is part of a nine-protein mortality risk prediction panel
PMID: 34226277
7
ITIH2 undergoes aging-associated structural changes in cerebrospinal fluid with relevance to neurodegeneration
PMID: 36741774
8
ITIH2 is present in plasma-derived extracellular vesicles and is associated with hepatocellular carcinoma patients
PMID: 39011654
Disease Associationsⓘ20
acute tonsillitisOpen Targets
0.35Weak
neurodegenerative diseaseOpen Targets
0.33Weak
type 2 diabetes mellitusOpen Targets
0.30Weak
non-alcoholic fatty liver diseaseOpen Targets
0.29Weak
adverse effectOpen Targets
0.28Weak
diaphragm diseaseOpen Targets
0.27Weak
lung cancerOpen Targets
0.08Suggestive
poisoningOpen Targets
0.05Suggestive
intracranial hemorrhageOpen Targets
0.05Suggestive
CachexiaOpen Targets
0.05Suggestive
response to antibioticOpen Targets
0.05Suggestive
Abnormal pupillary functionOpen Targets
0.04Suggestive
colorectal cancerOpen Targets
0.04Suggestive
endometrial cancerOpen Targets
0.04Suggestive
breast cancerOpen Targets
0.04Suggestive
neoplasmOpen Targets
0.03Suggestive
systemic lupus erythematosusOpen Targets
0.03Suggestive
rheumatoid arthritisOpen Targets
0.03Suggestive
cancerOpen Targets
0.03Suggestive
Huntington diseaseOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ITIH6Shared pathway100%ITIH5Shared pathway100%APOBProtein interaction99%APOA1Protein interaction99%F2Protein interaction98%ITIH1Protein interaction98%
Tissue Expression6 tissues
Liver
100%
Brain
0%
Lung
0%
Heart
0%
Ovary
0%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
ITIH2ITIH6ITIH5APOBAPOA1F2ITIH1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt P19823
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.21LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.99 [0.81–1.21]
RankingsWhere ITIH2 stands among ~20K protein-coding genes
  • #6,806of 20,598
    Most Researched69
  • #12,667of 17,882
    Most Constrained (LOEUF)1.21
Genes detectedITIH2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Hyaluronan network remodeling by ZEB1 and ITIH2 enhances the motility and invasiveness of cancer cells.
PMID: 40178908
J Clin Invest · 2025
1.00
2
Limited Proteolysis-Mass Spectrometry Reveals Aging-Associated Changes in Cerebrospinal Fluid Protein Abundances and Structures.
PMID: 36741774
Nat Aging · 2022
0.90
3
Plasma extracellular vesicle long RNA profiling identifies a diagnostic signature for the detection of pancreatic ductal adenocarcinoma.
PMID: 31562239
Gut · 2020
0.80
4
Two RFLPs in human inter-alpha-trypsin inhibitor heavy chain gene ITIH2 on chromosome 10.
PMID: 2474798
Nucleic Acids Res · 1989
0.70
5
An hypervariable polymorphism detected in the human inter-alpha-trypsin inhibitor heavy chain gene ITIH2.
PMID: 1690878
Nucleic Acids Res · 1990
0.60