PCNX2

Chr 1

pecanex 2

Also known as: PCNXL2

PCNX2 encodes a protein that may be involved in preventing tumorigenesis in colorectal tissue with high microsatellite instability. The gene is not highly constrained to loss-of-function variation and defects are associated with colorectal carcinomas rather than pediatric neurological disorders. Based on the available information, this gene does not appear to have established associations with inherited neurogenetic conditions in children.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 0.68
Clinical SummaryPCNX2
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
0.68LOEUF
pLI 0.000
Z-score 4.23
OE 0.54 (0.430.68)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.21Z-score
OE missense 0.90 (0.860.95)
1065 obs / 1181.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.54 (0.430.68)
00.351.4
Missense OE0.90 (0.860.95)
00.61.4
Synonymous OE1.01
01.21.6
LoF obs/exp: 54 / 99.5Missense obs/exp: 1065 / 1181.7Syn Z: -0.09
DN
0.6551th %ile
GOF
0.6932th %ile
LOF
0.4233th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
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

PCNX2 · 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 →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found