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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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COL9A1
collagen type IX alpha 1 chain
Chromosome 6 · 6q13
NCBI Gene: 1297Ensembl: ENSG00000112280.18HGNC: HGNC:2217UniProt: A0A804HIB6
65PubMed Papers
22Diseases
0Drugs
100Pathogenic Variants
RESEARCH IMPACT
Variant-Rich
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingextracellular matrixcarbohydrate bindingprotein homodimerization activityStickler syndromeepiphyseal dysplasia, multiple, 6connective tissue diseaseStickler syndrom, recessive
✦AI Summary

COL9A1 encodes the α1 chain of type IX collagen, a structural component of hyaline cartilage and the vitreous of the eye that contributes to extracellular matrix organization and tensile strength 1. The gene contains two promoter regions: an upstream promoter driving expression of a longer chain in cartilage, and a downstream promoter driving expression of a shorter chain in the cornea and vitreous 1. Chondrocyte-specific expression is regulated by SOX9, which binds to multiple SOX/Sry-binding sites within the proximal promoter region 1. During osteoarthritis, LSD1, a chr6 remodeling enzyme, negatively regulates COL9A1 expression through interaction with SOX9 at the gene promoter, contributing to cartilage matrix degradation 2. Clinically, elevated plasma COL9A1 levels are among the strongest predictors of osteoarthritis development, with hazard ratios of 1.54 (95% CI 1.48-1.61), and levels begin deviating from normal more than a decade before disease onset 3. Pathogenic variants in COL9A1 cause autosomal recessive Stickler syndrome, characterized by ocular, auditory, and skeletal abnormalities 4, and COL9A1 mutations are identified in short-stature patients with skeletal dysplasias 5. Gene-environment interactions between COL9A1 polymorphisms and maternal drinking influence susceptibility to congenital talipes equinovarus 6.

Sources cited
1
COL9A1 has two promoter regions; upstream drives cartilage-specific expression of longer chain; downstream drives corneal/vitreous expression; SOX9 regulates chondrocyte-specific expression by binding SOX/Sry-binding sites
PMID: 12399468
2
LSD1 negatively regulates COL9A1 expression in articular chondrocytes through interaction with SOX9; increased LSD1 in OA cartilage contributes to COL9A1 downregulation and matrix degradation
PMID: 32878268
3
Plasma COL9A1 is a top predictor of incident osteoarthritis (HR 1.54, 95% CI 1.48-1.61); levels deviate from normal >10 years before OA onset, useful for early detection
PMID: 39989277
4
Pathogenic variants in COL9A1 cause autosomal recessive Stickler syndrome with high myopia, sensorineural hearing loss, and variable retinal detachment
PMID: 35885918
5
COL9A1 mutations identified in short-stature patients with skeletal abnormalities; mutations are one category of collagenopathies affecting skeletal development
PMID: 35250876
6
COL9A1 polymorphism rs6455357 shows gene-environment interaction with maternal drinking affecting congenital talipes equinovarus susceptibility
PMID: 33372835
Disease Associationsⓘ22
Stickler syndromeOpen Targets
0.76Strong
epiphyseal dysplasia, multiple, 6Open Targets
0.69Moderate
connective tissue diseaseOpen Targets
0.44Moderate
Stickler syndrom, recessiveOpen Targets
0.41Moderate
hearing lossOpen Targets
0.41Moderate
multiple epiphyseal dysplasia due to collagen 9 anomalyOpen Targets
0.38Weak
Sensorineural hearing impairmentOpen Targets
0.38Weak
multiple epiphyseal dysplasiaOpen Targets
0.37Weak
osteoarthritisOpen Targets
0.35Weak
genetic disorderOpen Targets
0.34Weak
sensorineural hearing lossOpen Targets
0.34Weak
Retinal dystrophyOpen Targets
0.31Weak
multiple epiphyseal dysplasia, Beighton typeOpen Targets
0.16Weak
Hearing impairmentOpen Targets
0.16Weak
optic atrophyOpen Targets
0.14Weak
chondrocalcinosis 2Open Targets
0.08Suggestive
rheumatic diseaseOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.06Suggestive
placental retentionOpen Targets
0.06Suggestive
Angel-shaped phalango-epiphyseal dysplasiaOpen Targets
0.06Suggestive
Multiple epiphyseal dysplasia 6UniProt
Stickler syndrome 4UniProt
Pathogenic Variants100
NM_001851.6(COL9A1):c.9dup (p.Cys4fs)Pathogenic
not provided|Connective tissue disorder
★★☆☆2026→ Residue 4
NM_001851.6(COL9A1):c.812dup (p.Gly272_Glu273insTer)Pathogenic
COL9A1-related disorder|not provided
★★☆☆2026→ Residue 272
NM_001851.6(COL9A1):c.1411C>T (p.Arg471Ter)Pathogenic
not provided
★★☆☆2026→ Residue 471
NM_001851.6(COL9A1):c.14G>A (p.Trp5Ter)Pathogenic
not provided|Stickler syndrome, type 4;Epiphyseal dysplasia, multiple, 6|Stickler syndrome, type 4
★★☆☆2026→ Residue 5
NM_001851.6(COL9A1):c.171del (p.Phe57fs)Pathogenic
not provided
★★☆☆2025→ Residue 57
NM_001851.6(COL9A1):c.911del (p.Pro304fs)Pathogenic
Epiphyseal dysplasia, multiple, 6|not provided
★★☆☆2025→ Residue 304
NM_001851.6(COL9A1):c.902dup (p.Pro306fs)Pathogenic
not provided
★★☆☆2025→ Residue 306
NM_001851.6(COL9A1):c.188del (p.Phe63fs)Pathogenic
not provided
★★☆☆2025→ Residue 63
NM_001851.6(COL9A1):c.1120G>T (p.Glu374Ter)Pathogenic
not provided|COL9A1-related disorder
★★☆☆2025→ Residue 374
NM_001851.6(COL9A1):c.1178del (p.Pro393fs)Pathogenic
not provided|COL9A1-related disorder
★★☆☆2025→ Residue 393
NM_001851.6(COL9A1):c.1519C>T (p.Arg507Ter)Pathogenic
Stickler syndrome, type 4|not provided|Connective tissue disorder
★★☆☆2024→ Residue 507
NM_001851.6(COL9A1):c.733del (p.Leu245fs)Pathogenic
not provided|COL9A1-related disorder
★★☆☆2024→ Residue 245
NM_001851.6(COL9A1):c.1052C>A (p.Ser351Ter)Likely pathogenic
not provided|Stickler syndrome, type 4
★★☆☆2024→ Residue 351
NM_001851.6(COL9A1):c.1852C>T (p.Arg618Ter)Pathogenic
not provided
★☆☆☆2026→ Residue 618
NM_001851.6(COL9A1):c.401_407dup (p.Ser137fs)Pathogenic
not provided
★☆☆☆2026→ Residue 137
NM_001851.6(COL9A1):c.2068_2069del (p.Arg690fs)Pathogenic
Monogenic hearing loss
★☆☆☆2025→ Residue 690
NM_001851.6(COL9A1):c.1179del (p.Gly394fs)Pathogenic
not provided
★☆☆☆2025→ Residue 394
NM_001851.6(COL9A1):c.1342-2A>GLikely pathogenic
not provided
★☆☆☆2025
NM_001851.6(COL9A1):c.1030-2A>GLikely pathogenic
not provided
★☆☆☆2025
NM_001851.6(COL9A1):c.88+1G>ALikely pathogenic
not provided
★☆☆☆2025
View on ClinVar ↗
Related Genes
GP6Protein interaction98%ITGB6Protein interaction97%ITGA2Protein interaction97%ITGA3Protein interaction96%ITGA11Protein interaction94%MATN3Protein interaction91%
Tissue Expression6 tissues
Heart
100%
Brain
16%
Bone Marrow
2%
Liver
1%
Ovary
1%
Lung
0%
Gene Interaction Network
Click a node to explore
COL9A1GP6ITGB6ITGA2ITGA3ITGA11MATN3
PROTEIN STRUCTURE
Preparing viewer…
PDB5CTD · 1.60 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.89LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.75 [0.63–0.89]
RankingsWhere COL9A1 stands among ~20K protein-coding genes
  • #7,156of 20,598
    Most Researched65
  • #778of 5,498
    Most Pathogenic Variants100 · top quartile
  • #7,961of 17,882
    Most Constrained (LOEUF)0.89
Genes detectedCOL9A1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
PMID: 20301479
1.00
2
Plasma Proteomic Profiles Predict Individual Future Osteoarthritis Risk.
PMID: 39989277
Arthritis Rheumatol · 2025
0.90
3
Data-driven identification of predictive risk biomarkers for subgroups of osteoarthritis using interpretable machine learning.
PMID: 38561399
Nat Commun · 2024
0.80
4
Autosomal Recessive Stickler Syndrome.
PMID: 35885918
Genes (Basel) · 2022
0.70
5
Clinical Characteristics of Short-Stature Patients With Collagen Gene Mutation and the Therapeutic Response to rhGH.
PMID: 35250876
Front Endocrinol (Lausanne) · 2022
0.60