MSMO1

Chr 4AR

methylsterol monooxygenase 1

Also known as: DESP4, ERG25, MCCPD, SC4MOL

Sterol-C4-mehtyl oxidase-like protein was isolated based on its similarity to the yeast ERG25 protein. It contains a set of putative metal binding motifs with similarity to that seen in a family of membrane desaturases-hydroxylases. The protein is localized to the endoplasmic reticulum membrane and is believed to function in cholesterol biosynthesis. Alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

Primary Disease Associations & Inheritance

Microcephaly, congenital cataract, and psoriasiform dermatitisMIM #616834
AR
0
Active trials
21
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.70
LOEUF
DN
Mechanism· predicted
Clinical SummaryMSMO1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.31) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.70LOEUF
pLI 0.090
Z-score 2.32
OE 0.31 (0.150.70)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.59Z-score
OE missense 0.86 (0.751.00)
129 obs / 149.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.31 (0.150.70)
00.351.4
Missense OE0.86 (0.751.00)
00.61.4
Synonymous OE0.74
01.21.6
LoF obs/exp: 4 / 13.0Missense obs/exp: 129 / 149.3Syn Z: 1.42
DN
0.6938th %ile
GOF
0.6247th %ile
LOF
0.2968th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MSMO1 · 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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