PPM1A

Chr 14

protein phosphatase, Mg2+/Mn2+ dependent 1A

Also known as: PP2C-ALPHA, PP2CA, PP2Calpha

PPM1A encodes a serine/threonine protein phosphatase that negatively regulates multiple cellular signaling pathways including TGF-beta, NF-kappa-B, and AMPK signaling through dephosphorylation of key regulatory proteins. Mutations cause autosomal dominant intellectual disability with developmental delay, and the gene is highly intolerant to loss-of-function variants. The phenotype typically manifests in early childhood with cognitive impairment as the primary feature.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.16
Clinical SummaryPPM1A
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.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.16LOEUF
pLI 0.998
Z-score 4.00
OE 0.00 (0.000.16)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
2.58Z-score
OE missense 0.53 (0.460.62)
127 obs / 239.1 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.00 (0.000.16)
00.351.4
Missense OE0.53 (0.460.62)
00.61.4
Synonymous OE0.94
01.21.6
LoF obs/exp: 0 / 18.7Missense obs/exp: 127 / 239.1Syn Z: 0.42
DN
0.3793th %ile
GOF
0.5268th %ile
LOF
0.67top 25%

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

LOFprediction above median · LOEUF 0.16

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

PPM1A · 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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