DGAT1

Chr 8AR

diacylglycerol O-acyltransferase 1

Also known as: ARAT, ARGP1, DGAT, DIAR7

The protein catalyzes the terminal step in triacylglycerol synthesis and is highly expressed in small intestinal epithelial cells where it is essential for dietary fat absorption. Mutations cause autosomal recessive diarrhea 7 with protein-losing enteropathy. The gene is not highly constrained against loss-of-function variants, consistent with the recessive inheritance pattern.

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

Diarrhea 7, protein-losing enteropathy typeMIM #615863
AR
0
Active trials
130
Pubs (1 yr)
83
P/LP submissions
1%
P/LP missense
1.22
LOEUF
LOF
Mechanism· G2P
Clinical SummaryDGAT1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
73 unique Pathogenic / Likely Pathogenic· 142 VUS of 600 total submissions
📖
GeneReview available — DGAT1
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.22LOEUF
pLI 0.000
Z-score 0.58
OE 0.89 (0.661.22)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
1.04Z-score
OE missense 0.82 (0.740.92)
229 obs / 277.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.89 (0.661.22)
00.351.4
Missense OE0.82 (0.740.92)
00.61.4
Synonymous OE1.13
01.21.6
LoF obs/exp: 28 / 31.5Missense obs/exp: 229 / 277.9Syn Z: -1.11
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedDGAT1-related congenital diarrheal disorderLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6842th %ile
GOF
0.7029th %ile
LOF
0.3550th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

600 submitted variants in ClinVar

Classification Summary

Pathogenic48
Likely Pathogenic25
VUS142
Likely Benign336
Benign23
Conflicting8
48
Pathogenic
25
Likely Pathogenic
142
VUS
336
Likely Benign
23
Benign
8
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
24
0
24
0
48
Likely Pathogenic
19
1
5
0
25
VUS
2
116
24
0
142
Likely Benign
0
2
182
152
336
Benign
0
1
14
8
23
Conflicting
8
Total45120249160582

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

View in ClinVar →

Protein Context — Lollipop Plot

DGAT1 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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