ITGB1

Chr 10

integrin subunit beta 1

Also known as: CD29, FNRB, GPIIA, MDF2, MSK12, VLA-BETA, VLAB

The ITGB1 protein forms heterodimeric integrin receptors with various alpha subunits that mediate cell adhesion to extracellular matrix components including collagen, fibronectin, and laminin, playing critical roles in cell migration, tissue development, and vascular function. Mutations cause autosomal recessive congenital glanzmann thrombasthenia-like bleeding disorder and Ehlers-Danlos syndrome with bleeding diathesis, affecting hemostasis and connective tissue integrity. This gene is highly constrained against loss-of-function variants (pLI = 0.98), indicating that biallelic mutations are likely required for disease manifestation.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
248
Pubs (1 yr)
10
P/LP submissions
0%
P/LP missense
0.31
LOEUF· LoF intol.
Mechanism
Clinical SummaryITGB1
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.98). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
10 unique Pathogenic / Likely Pathogenic· 70 VUS of 113 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.31LOEUF
pLI 0.982
Z-score 4.87
OE 0.15 (0.080.31)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
3.46Z-score
OE missense 0.53 (0.470.59)
227 obs / 428.5 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.15 (0.080.31)
00.351.4
Missense OE0.53 (0.470.59)
00.61.4
Synonymous OE0.95
01.21.6
LoF obs/exp: 6 / 38.7Missense obs/exp: 227 / 428.5Syn Z: 0.50

ClinVar Variant Classifications

113 submitted variants in ClinVar

Classification Summary

Pathogenic10
VUS70
Likely Benign6
Benign5
10
Pathogenic
70
VUS
6
Likely Benign
5
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
10
0
10
Likely Pathogenic
0
0
0
0
0
VUS
0
63
7
0
70
Likely Benign
0
2
2
2
6
Benign
0
0
3
2
5
Total06522491

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

ITGB1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
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