IDI2

Chr 10

isopentenyl-diphosphate delta isomerase 2

Also known as: IPPI2

The protein catalyzes the conversion of isopentenyl diphosphate to dimethylallyl diphosphate, a key step in cholesterol and isoprenoid biosynthesis. Mutations cause sterol biosynthesis defect with corpus callosum agenesis, cataracts, and growth restriction, inherited in an autosomal recessive pattern. This gene is not highly constrained against loss-of-function variants, which is consistent with the recessive inheritance pattern observed clinically.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.94
Clinical SummaryIDI2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.94LOEUF
pLI 0.000
Z-score -1.34
OE 1.54 (0.931.94)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.56Z-score
OE missense 1.13 (0.991.30)
153 obs / 134.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.54 (0.931.94)
00.351.4
Missense OE1.13 (0.991.30)
00.61.4
Synonymous OE1.22
01.21.6
LoF obs/exp: 11 / 7.1Missense obs/exp: 153 / 134.8Syn Z: -1.30

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

IDI2 · 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
Open Research Assistant →