TMEM98

Chr 17AD

transmembrane protein 98

Also known as: TADA1

This transmembrane protein functions as a negative regulator of MYRF in oligodendrocyte differentiation and myelination by inhibiting MYRF self-cleavage and nuclear translocation, and its secreted form promotes Th1 cell differentiation. Missense mutations cause nanophthalmos 4, an autosomal dominant condition characterized by abnormally small eyes. The pathogenic mechanism involves gain-of-function mutations.

Summary from RefSeq, OMIM, UniProt, Mechanism
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Primary Disease Associations & Inheritance

Nanophthalmos 4MIM #615972
AD
0
Active trials
6
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.89
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryTMEM98
🧬
Gene-Disease Validity (ClinGen)
nanophthalmos 4 · ADLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Low constraint (pLI 0.01) — 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
0.89LOEUF
pLI 0.012
Z-score 1.85
OE 0.42 (0.220.89)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.73Z-score
OE missense 0.82 (0.700.96)
110 obs / 133.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.42 (0.220.89)
00.351.4
Missense OE0.82 (0.700.96)
00.61.4
Synonymous OE0.97
01.21.6
LoF obs/exp: 5 / 11.9Missense obs/exp: 110 / 133.7Syn Z: 0.15
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongTMEM98-related nanophthalmosOTHERAD
DN
0.6162th %ile
GOF
0.6930th %ile
LOF
0.3067th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
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

TMEM98 · 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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