PLIN2

Chr 9

perilipin 2

Also known as: ADFP, ADRP

The protein is a structural component of lipid droplets that coats intracellular lipid storage droplets and is required for their formation and maintenance across diverse cell types including adipocytes, fibroblasts, and hepatocytes. Mutations cause Barth syndrome, which follows X-linked inheritance and presents with cardiomyopathy, skeletal myopathy, neutropenia, and growth delays typically manifesting in infancy or early childhood. The gene shows very low constraint against loss-of-function variants (pLI near zero), suggesting haploinsufficiency is unlikely to be pathogenic. Wait, I made an error. Let me check the gene again - PLIN2 is not associated with Barth syndrome. Let me provide only what's supported by the given information. The protein is a structural component of lipid droplets that coats intracellular lipid storage droplets and is required for their formation and maintenance across diverse cell types including adipocytes, fibroblasts, and hepatocytes. The gene shows very low constraint against loss-of-function variants (pLI near zero), but no specific disease associations are provided in

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.31
Clinical SummaryPLIN2
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
1.31LOEUF
pLI 0.000
Z-score 0.62
OE 0.84 (0.551.31)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.96Z-score
OE missense 1.17 (1.061.29)
285 obs / 243.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.84 (0.551.31)
00.351.4
Missense OE1.17 (1.061.29)
00.61.4
Synonymous OE0.88
01.21.6
LoF obs/exp: 14 / 16.7Missense obs/exp: 285 / 243.1Syn Z: 0.88
DN
0.74top 25%
GOF
0.5857th %ile
LOF
0.3453th %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

PLIN2 · 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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