TAMM41

Chr 3AR

TAM41 mitochondrial translocator assembly and maintenance homolog

Also known as: C3orf31, COXPD56, RAM41, TAM41

TAMM41 encodes a mitochondrial enzyme that catalyzes the conversion of phosphatidic acid to CDP-diacylglycerol, an essential step in cardiolipin biosynthesis. Mutations cause combined oxidative phosphorylation deficiency 56, a mitochondrial disorder affecting cellular energy production. This condition follows autosomal recessive inheritance.

OMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismARLOEUF 1.061 OMIM phenotype
Clinical SummaryTAMM41
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
1.06LOEUF
pLI 0.000
Z-score 1.37
OE 0.65 (0.421.06)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.01Z-score
OE missense 1.00 (0.881.14)
172 obs / 171.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.65 (0.421.06)
00.351.4
Missense OE1.00 (0.881.14)
00.61.4
Synonymous OE1.16
01.21.6
LoF obs/exp: 12 / 18.3Missense obs/exp: 172 / 171.8Syn Z: -1.03
DN
0.6357th %ile
GOF
0.5857th %ile
LOF
0.3647th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction 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

TAMM41 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

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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