PLPP7

Chr 9

phospholipid phosphatase 7 (inactive)

Also known as: C9orf67, NET39, PPAPDC3

The protein acts as a negative regulator of myoblast differentiation through effects on MTOR signaling and is located in the endoplasmic reticulum membrane and nucleus. Mutations in PLPP7 cause autosomal recessive intellectual disability with microcephaly and seizures, typically presenting in infancy or early childhood. This gene affects neurodevelopment and muscle differentiation pathways.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.32
Clinical SummaryPLPP7
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
1.32LOEUF
pLI 0.008
Z-score 1.01
OE 0.58 (0.291.32)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.73Z-score
OE missense 0.85 (0.740.97)
152 obs / 179.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.58 (0.291.32)
00.351.4
Missense OE0.85 (0.740.97)
00.61.4
Synonymous OE0.92
01.21.6
LoF obs/exp: 4 / 6.9Missense obs/exp: 152 / 179.5Syn Z: 0.54
DN
0.6648th %ile
GOF
0.5269th %ile
LOF
0.4429th %ile

The highest-scoring mechanism for this gene is dominant-negative.

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

PLPP7 · 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.

Search ClinicalTrials.gov →