The protein catalyzes the CTP-mediated phosphorylation of dolichol to synthesize Dol-P-Man, an essential glycosyl carrier lipid required for protein glycosylation and glycosyl phosphatidylinositol anchor biosynthesis in the endoplasmic reticulum. Mutations cause congenital disorder of glycosylation type Im through autosomal recessive inheritance. The pathogenic mechanism involves dominant-negative effects of mutant protein.

OMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismARLOEUF 0.901 OMIM phenotype
Clinical SummaryDOLK
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Gene-Disease Validity (ClinGen)
DK1-congenital disorder of glycosylation · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.90LOEUF
pLI 0.001
Z-score 1.85
OE 0.48 (0.270.90)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.98Z-score
OE missense 0.84 (0.760.93)
251 obs / 298.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.48 (0.270.90)
00.351.4
Missense OE0.84 (0.760.93)
00.61.4
Synonymous OE1.09
01.21.6
LoF obs/exp: 7 / 14.6Missense obs/exp: 251 / 298.5Syn Z: -0.84
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveDOLK-related congenital disorder of glycosylationLOFAR

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

DN
0.6939th %ile
GOF
0.4973th %ile
LOF
0.2580th %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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DOLK · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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