MROH6

Chr 8

maestro heat like repeat family member 6

Also known as: C8orf73

The MROH6 protein is predicted to be active in the cytoplasm, but its specific cellular function remains unclear. Mutations in this gene have been associated with neurodevelopmental disorders, though the clinical phenotype and inheritance pattern are not well-established from current data. This gene appears to tolerate loss-of-function variants well based on constraint metrics.

Summary from RefSeq
Research Assistant →
0
Active trials
2
Pubs (1 yr)
61
P/LP submissions
0%
P/LP missense
1.35
LOEUF
GOF
Mechanism· predicted
Clinical SummaryMROH6
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
60 unique Pathogenic / Likely Pathogenic· 203 VUS of 302 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.35LOEUF
pLI 0.000
Z-score 0.09
OE 0.98 (0.721.35)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.66Z-score
OE missense 1.10 (1.011.19)
402 obs / 366.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.98 (0.721.35)
00.351.4
Missense OE1.10 (1.011.19)
00.61.4
Synonymous OE1.15
01.21.6
LoF obs/exp: 27 / 27.5Missense obs/exp: 402 / 366.4Syn Z: -1.56
DN
0.6065th %ile
GOF
0.7127th %ile
LOF
0.3453th %ile

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction 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

302 submitted variants in ClinVar

Classification Summary

Pathogenic55
Likely Pathogenic5
VUS203
Likely Benign15
55
Pathogenic
5
Likely Pathogenic
203
VUS
15
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
55
0
55
Likely Pathogenic
0
0
5
0
5
VUS
0
198
5
0
203
Likely Benign
0
14
0
1
15
Benign
0
0
0
0
0
Total0212651278

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

View in ClinVar →

Protein Context — Lollipop Plot

MROH6 · 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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