MGME1

Chr 20

mitochondrial genome maintenance exonuclease 1

Also known as: C20orf72, DDK1, MTDPS11, bA504H3.4

The protein encoded by this gene is a nuclear-encoded mitochondrial protein necessary for the maintenance of mitochondrial genome synthesis. The encoded protein is a RecB-type exonuclease and primarily cleaves single-stranded DNA. Defects in this gene have been associated with mitochondrial DNA depletion syndrome-11. Three transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2015]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtMitochondrial DNA depletion syndrome 11

Clinical highlights

Gene-disease validity (ClinGen)
mitochondrial disease · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
10
Pubs (1 yr)
P/LP submissions
P/LP missense
0.84
LOEUF
DN
Mechanism· predicted
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?
0.84LOEUF
pLI 0.002
Z-score 2.03
OE 0.45 (0.250.84)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.26Z-score
OE missense 1.05 (0.941.19)
187 obs / 177.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.45 (0.250.84)
00.351.4
Missense OE?1.05 (0.941.19)
00.61.4
Synonymous OE?0.89
01.21.6
LoF obs/exp: 7 / 15.6Missense obs/exp: 187 / 177.3Syn Z: 0.67

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MGME1 · 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 →