MYT1L

Chr 2AD

myelin transcription factor 1 like

Also known as: MRD39, NZF1, ZC2H2C2, ZC2HC4B, myT1-L

This gene encodes a zinc finger transcription factor that functions in developing mammalian central nervous system and can convert fibroblasts into induced neuronal cells capable of generating action potentials. Loss-of-function mutations cause autosomal dominant intellectual developmental disorder and autism spectrum disorder. The gene is extremely intolerant to loss-of-function variation, supporting haploinsufficiency as the mechanism of pathogenicity.

OMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismADLOEUF 0.091 OMIM phenotype
Clinical SummaryMYT1L
🧬
Gene-Disease Validity (ClinGen)
syndromic complex neurodevelopmental disorder · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
💊
Clinical Trials
3 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.09LOEUF
pLI 1.000
Z-score 6.71
OE 0.02 (0.010.09)
Highly constrained

Among the most LoF-intolerant genes (~top 3%)

Missense Constraint
4.77Z-score
OE missense 0.50 (0.460.55)
365 obs / 727.0 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.02 (0.010.09)
00.351.4
Missense OE0.50 (0.460.55)
00.61.4
Synonymous OE0.84
01.21.6
LoF obs/exp: 1 / 54.4Missense obs/exp: 365 / 727.0Syn Z: 2.25
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveMYT1L-related intellectual developmental syndromeLOFAD
DN
0.2399th %ile
GOF
0.1899th %ile
LOF
0.87top 5%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · 1 literature citation · LOEUF 0.09

Literature Evidence

LOFMoreover, because 17 patients present with obesity/overweight, haploinsufficiency of MYT1L might predispose to weight problems with childhood onset.PMID:25232846

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MYT1L · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →
Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗