PSAT1

Chr 9AR

phosphoserine aminotransferase 1

Also known as: EPIP, NLS2, PSA, PSAT, PSATD

This gene encodes a member of the class-V pyridoxal-phosphate-dependent aminotransferase family. The encoded protein is a phosphoserine aminotransferase and decreased expression may be associated with schizophrenia. Mutations in this gene are also associated with phosphoserine aminotransferase deficiency. Alternative splicing results in multiple transcript variants. Pseudogenes of this gene have been defined on chromosomes 1, 3, and 8. [provided by RefSeq, Jul 2013]

OMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 1.272 OMIM phenotypes
Clinical SummaryPSAT1
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Gene-Disease Validity (ClinGen)
neurometabolic disorder due to serine deficiency · 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.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.27LOEUF
pLI 0.000
Z-score 0.71
OE 0.81 (0.541.27)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.41Z-score
OE missense 0.92 (0.811.04)
185 obs / 201.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.81 (0.541.27)
00.351.4
Missense OE?0.92 (0.811.04)
00.61.4
Synonymous OE?0.93
01.21.6
LoF obs/exp: 14 / 17.2Missense obs/exp: 185 / 201.5Syn Z: 0.50
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongPSAT1-related Neu-Laxova syndromeLOFAR

This gene — mechanism propensity

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

DN
0.6161th %ile
GOF
0.4480th %ile
LOF
0.3746th %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

PSAT1 · 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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