AMT

Chr 3

aminomethyltransferase

Also known as: GCE, GCE2, GCST, GCVT, NKH

This gene encodes one of four critical components of the glycine cleavage system. Mutations in this gene have been associated with glycine encephalopathy. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2011]

GeneReviewsResearchGenerating clinical summary…
LOFmechanismLOEUF 0.79
Clinical SummaryAMT
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Gene-Disease Validity (ClinGen)
glycine encephalopathy · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
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GeneReview available — AMT
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
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?
0.79LOEUF
pLI 0.003
Z-score 2.19
OE 0.42 (0.240.79)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.10Z-score
OE missense 1.02 (0.921.13)
244 obs / 239.6 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.42 (0.240.79)
00.351.4
Missense OE?1.02 (0.921.13)
00.61.4
Synonymous OE?1.06
01.21.6
LoF obs/exp: 7 / 16.7Missense obs/exp: 244 / 239.6Syn Z: -0.47
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveAMT-related glycine encephalopathyLOFAR

This gene — mechanism propensity

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.74top 25%
GOF
0.73top 25%
LOF
0.3453th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

AMT · protein map & ClinVar variants

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