MPV17

Chr 2

mitochondrial inner membrane protein MPV17

Non-selective channel that modulates the membrane potential under normal conditions and oxidative stress, and is involved in mitochondrial homeostasis (PubMed:25861990). Involved in mitochondrial deoxynucleoside triphosphates (dNTP) pool homeostasis and mitochondrial DNA (mtDNA) maintenance (PubMed:26760297). May be involved in the regulation of reactive oxygen species metabolism and the control of oxidative phosphorylation (By similarity)

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtMitochondrial DNA depletion syndrome 6
UniProtCharcot-Marie-Tooth disease, axonal, type 2EE

Clinical highlights

Gene-disease validity (ClinGen)
mitochondrial disease · ARDefinitivesufficient evidence for diagnostic panels
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype), though the gene is not strongly LoF-constrained in the population — weigh truncating variants against that tolerance.Curated gene-level mechanism — a prior for triage, not a per-variant call.
8
Active trials
15
Pubs (1 yr)
P/LP submissions
P/LP missense
1.79
LOEUF
LOF
Mechanism· G2P
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?
1.79LOEUF
pLI 0.000
Z-score -0.65
OE 1.20 (0.791.79)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.24Z-score
OE missense 0.93 (0.781.11)
89 obs / 95.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?1.20 (0.791.79)
00.351.4
Missense OE?0.93 (0.781.11)
00.61.4
Synonymous OE?0.95
01.21.6
LoF obs/exp: 14 / 11.6Missense obs/exp: 89 / 95.5Syn Z: 0.26

ClinVar

No ClinVar data available.

Protein Context — Lollipop Plot

MPV17 · 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

Mitochondrial DiseasesMitochondrial EncephalomyopathyMitochondrial Encephalopathy

Deoxynucleosides Pyrimidines as Treatment for Mitochondrial Depletion Syndrome

RECRUITING
NCT04802707Phase PHASE2Kenneth Myers, MDStarted 2021-10-18
deoxycytidine and deoxythymidine
ANCA Associated VasculitisExtramembranous GlomerulopathyNephrotic Syndrome, Minimal Change

Epithelial Dysmetabolism and Renal Fibrosis in ANCA Vasculitis

RECRUITING
NCT07010250Assistance Publique - Hôpitaux de ParisStarted 2025-11-03
Focal Segmental GlomerulosclerosisNephrotic SyndromeEnd Stage Renal Disease

Genetic Causes of FSGS, Nephrotic Syndrome, or Kidney Failure

ACTIVE NOT RECRUITING
NCT02194582Beth Israel Deaconess Medical CenterStarted 1996-06
Adenine Phosphoribosyltransferase DeficiencyAH AmyloidosisAHL Amyloidosis

National Registry of Rare Kidney Diseases

RECRUITING
NCT06065852UK Kidney AssociationStarted 2009-11-06
HIV NephropathyKidney InjuryKidney Diseases

Genetic Determinants of Kidney Disease in People of African Ancestry With HIV

ACTIVE NOT RECRUITING
NCT05685810King's College Hospital NHS TrustStarted 2018-05-01
Focal Segmental Glomerulosclerosis

A Study to Find Out if BI 764198 Helps Adults and Adolescents With a Kidney Condition Called Focal Segmental Glomerulosclerosis (FSGS)

RECRUITING
NCT07220083Phase PHASE3Boehringer IngelheimStarted 2026-02-16
BI 764198Placebo
Nephrotic Syndrome

INSIGHT (Insight Into Nephrotic Syndrome)

RECRUITING
NCT01605266The Hospital for Sick ChildrenStarted 2011-01
Transplant ComplicationKidney TransplantLiver Transplant

"A Privacy-protecting Environment for Child Transplants Health Related and Genomic Data Integration in the European Reference Network"

NOT YET RECRUITING
NCT07194057Instituto de Investigación Hospital Universitario La PazStarted 2025-09-30
Whole genome sequencingPolygenic Risk Score CalculationMethylome and episignatures