MOCOS

Chr 18

molybdenum cofactor sulfurase

Also known as: HMCS, MCS, MOS

This gene encodes an enzyme that sulfurates the molybdenum cofactor which is required for activation of the xanthine dehydrogenase (XDH) and aldehyde oxidase (AO) enzymes. XDH catalyzes the conversion of hypoxanthine to uric acid via xanthine, as well as the conversion of allopurinol to oxypurinol, and pyrazinamide to 5-hydroxy pyrazinamide. Mutations in this gene cause the metabolic disorder classical xanthinuria type II which is characterized by the loss of XDH/XO and AO enzyme activity, decreased levels of uric acid in the urine, increased levels of xanthine and hypoxanthine in the serum and urine, formation of xanthine stones in the urinary tract, and myositis due to tissue deposition of xanthine. [provided by RefSeq, Apr 2017]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtXanthinuria 2

Clinical highlights

Gene-disease validity (ClinGen)
xanthinuria type II · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
14
Pubs (1 yr)
P/LP submissions
P/LP missense
1.15
LOEUF
Mechanism
📖
GeneReview available — MOCOS
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.15LOEUF
pLI 0.000
Z-score 0.80
OE 0.86 (0.651.15)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.45Z-score
OE missense 1.06 (0.981.14)
503 obs / 475.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.86 (0.651.15)
00.351.4
Missense OE?1.06 (0.981.14)
00.61.4
Synonymous OE?1.02
01.21.6
LoF obs/exp: 32 / 37.2Missense obs/exp: 503 / 475.4Syn Z: -0.17

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MOCOS · 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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