ANGPT1

Chr 8AD

angiopoietin 1

Also known as: AGP1, AGPT, AGPT-1, ANG1, HAE5

This gene encodes a secreted glycoprotein that belongs to the angiopoietin family. Members of this family play important roles in vascular development and angiogenesis. All angiopoietins bind with similar affinity to an endothelial cell-specific tyrosine-protein kinase receptor. The protein encoded by this gene is a secreted glycoprotein that activates the receptor by inducing its tyrosine phosphorylation. It plays a critical role in mediating reciprocal interactions between the endothelium and surrounding matrix and mesenchyme and inhibits endothelial permeability. The protein also contributes to blood vessel maturation and stability, and may be involved in early development of the heart. Mutations in this gene are associated with hereditary angioedema. [provided by RefSeq, Aug 2020]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

?Angioedema, hereditary, 5MIM #619361
AD

Clinical highlights

Gene-disease validity (ClinGen)
primary congenital glaucoma · ADLimitednot for standalone diagnostic reporting
Interpreting a novel variant
This gene is strongly intolerant of loss-of-function variation in the population, so LoF variants warrant close attention. No curated mechanism annotation is available — see the mechanism card for the computational prediction and its caveats.Based on population constraint only.
3
Active trials
96
Pubs (1 yr)
P/LP submissions
P/LP missense
0.34
LOEUF· LoF intol.
LOF
Mechanism· predicted
📖
GeneReview available — ANGPT1
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint?
0.34LOEUF
pLI 0.952
Z-score 4.12
OE 0.15 (0.070.34)
Highly constrained

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

Missense Constraint?
1.30Z-score
OE missense 0.77 (0.690.87)
202 obs / 261.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.15 (0.070.34)
00.351.4
Missense OE?0.77 (0.690.87)
00.61.4
Synonymous OE?0.93
01.21.6
LoF obs/exp: 4 / 27.2Missense obs/exp: 202 / 261.2Syn Z: 0.51

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ANGPT1 · protein map & ClinVar variants

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