PIANP

Chr 12

PILR alpha associated neural protein

Also known as: C12orf53, LEDA1, PANP, leda-1

PIANP encodes a ligand for the paired immunoglobulin-like type 2 receptor alpha that functions in neural immune regulation. Mutations cause neurodevelopmental disorder with progressive microcephaly, spasticity, and brain atrophy, inherited in an autosomal recessive pattern. The gene shows moderate constraint against loss-of-function variants.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
1
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.58
LOEUF
GOF
Mechanism· predicted
Clinical SummaryPIANP
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.22) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.58LOEUF
pLI 0.378
Z-score 2.64
OE 0.22 (0.100.58)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.95Z-score
OE missense 0.79 (0.680.91)
127 obs / 160.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.22 (0.100.58)
00.351.4
Missense OE0.79 (0.680.91)
00.61.4
Synonymous OE0.85
01.21.6
LoF obs/exp: 3 / 13.5Missense obs/exp: 127 / 160.9Syn Z: 0.95
DN
0.5181th %ile
GOF
0.6834th %ile
LOF
0.54top 25%

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction 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

PIANP · 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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Clinical Literature
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Key Publications
Landmark & review papers · by relevance
PubMed
Top 2 results · since 2015Search PubMed ↗