DHCR24

Chr 1AR

24-dehydrocholesterol reductase

Also known as: DCE, Nbla03646, SELADIN1, seladin-1

The protein is a FAD-dependent oxidoreductase that catalyzes the reduction of the delta-24 double bond of sterol intermediates during cholesterol biosynthesis in the endoplasmic reticulum. Mutations cause desmosterolosis, a cholesterol biosynthesis disorder that typically presents in infancy with developmental delay, seizures, and distinctive facial features. The condition follows autosomal recessive inheritance.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.521 OMIM phenotype
Clinical SummaryDHCR24
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Gene-Disease Validity (ClinGen)
desmosterolosis · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.29) despite low pLI — interpret in context.
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Clinical Trials
2 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.52LOEUF
pLI 0.031
Z-score 3.46
OE 0.29 (0.170.52)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.50Z-score
OE missense 0.76 (0.680.85)
232 obs / 305.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.29 (0.170.52)
00.351.4
Missense OE0.76 (0.680.85)
00.61.4
Synonymous OE0.93
01.21.6
LoF obs/exp: 8 / 27.6Missense obs/exp: 232 / 305.9Syn Z: 0.65

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DHCR24 · 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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