MIGA2

Chr 9

mitoguardin 2

Also known as: C9orf54, FAM73B

The protein regulates mitochondrial fusion by forming homo- and heterodimers at the mitochondrial outer membrane and facilitating PLD6/MitoPLD dimer formation, potentially through regulation of phospholipid metabolism. Mutations are predicted to cause disease through a gain-of-function mechanism. The gene shows tolerance to loss-of-function variants, suggesting haploinsufficiency is unlikely to be pathogenic.

Summary from RefSeq, UniProt, Mechanism
0
Active trials
0
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.67
LOEUF
GOF
Mechanism· predicted
Clinical SummaryMIGA2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.67LOEUF
pLI 0.000
Z-score 2.99
OE 0.42 (0.270.67)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.12Z-score
OE missense 0.84 (0.760.92)
316 obs / 376.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.42 (0.270.67)
00.351.4
Missense OE0.84 (0.760.92)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 13 / 31.0Missense obs/exp: 316 / 376.9Syn Z: 0.06
DN
0.5771th %ile
GOF
0.6736th %ile
LOF
0.3842th %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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MIGA2 · 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
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗