MINDY3

Chr 10

MINDY lysine 48 deubiquitinase 3

Also known as: C10orf97, CARP, DERP5, FAM188A, MST126, MSTP126, my042

The protein functions as a hydrolase that removes Lys-48-linked ubiquitin from proteins and contains a caspase-associated recruitment domain that may function in apoptosis. Mutations cause neurodevelopmental disorders with intellectual disability and developmental delay. The gene shows autosomal recessive inheritance and is moderately constrained against loss-of-function variants.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
1
Pubs (1 yr)
15
P/LP submissions
0%
P/LP missense
0.54
LOEUF
Mechanism
Clinical SummaryMINDY3
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.31) despite low pLI — interpret in context.
📋
ClinVar Variants
15 unique Pathogenic / Likely Pathogenic· 65 VUS of 108 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.54LOEUF
pLI 0.011
Z-score 3.47
OE 0.31 (0.180.54)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.60Z-score
OE missense 0.89 (0.791.00)
203 obs / 228.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.31 (0.180.54)
00.351.4
Missense OE0.89 (0.791.00)
00.61.4
Synonymous OE0.94
01.21.6
LoF obs/exp: 9 / 29.2Missense obs/exp: 203 / 228.5Syn Z: 0.42

ClinVar Variant Classifications

108 submitted variants in ClinVar

Classification Summary

Pathogenic15
VUS65
Likely Benign5
Benign6
15
Pathogenic
65
VUS
5
Likely Benign
6
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
15
0
15
Likely Pathogenic
0
0
0
0
0
VUS
0
58
7
0
65
Likely Benign
0
2
3
0
5
Benign
0
0
5
1
6
Total06030191

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

MINDY3 · 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.

Search ClinicalTrials.gov →
Clinical Literature
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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found