MOCOS

Chr 18q12.2

molybdenum cofactor sulfurase

Aliases:: HMCS, FLJ20733, MOS, MCS
MANE:: ENST00000261326.6

OMIM: 613274 ↗NCBI: 55034 ↗Ensembl ↗

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Predicted protein structure

AlphaFold predicted structure

MOCOS · Q96EN8

v62025-08-01

Predicted Aligned Error matrix for MOCOS — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

80.0/ 100

Confident

888 residues

Confidence breakdown

Very high(≥ 90)57%
Confident(70–90)19%
Low(50–70)6%
Very low(< 50)17%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Diagnostic Grade (Green)

Nephrocalcinosis or nephrolithiasis
BIALLELIC, autosomal or pseudoautosomal

Disease associations (Open Targets)

xanthinuria type II
0.78
hereditary xanthinuria
0.51
autism spectrum disorder
0.33
stricture
0.26
pathological myopia
0.20
primary hyperoxaluria
0.13
familial idiopathic steroid-resistant nephrotic syndrome
0.07
nephronophthisis
0.06
Hyperlipoproteinemia type 1
0.06
Dent disease
0.06

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

Molybdenum cofactor sulfurase

Sulfurates the molybdenum cofactor (PubMed:34356852). Sulfation of molybdenum is essential for xanthine dehydrogenase (XDH) and aldehyde oxidase (ADO) enzymes in which molybdenum cofactor is liganded by 1 oxygen and 1 sulfur atom in active form (PubMed:34356852). In vitro, the C-terminal domain is able to reduce N-hydroxylated prodrugs, such as benzamidoxime (PubMed:16973608)