PEX12

Chr 17AR

peroxisomal biogenesis factor 12

Also known as: PAF-3, PBD3A

PEX12 encodes a peroxisome membrane protein essential for the assembly of functional peroxisomes and the import of peroxisomal matrix proteins into the organelle. Mutations cause autosomal recessive peroxisome biogenesis disorders including Zellweger syndrome (PBD3A) and PBD3B, which are lethal conditions characterized by multiple defects in peroxisome function. The pathogenic mechanism involves defective import of peroxisomal matrix proteins due to impaired peroxisome assembly.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.852 OMIM phenotypes
Clinical SummaryPEX12
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Gene-Disease Validity (ClinGen)
peroxisome biogenesis disorder · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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GeneReview available — PEX12
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.85LOEUF
pLI 0.000
Z-score -1.23
OE 1.34 (0.941.85)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.46Z-score
OE missense 0.90 (0.791.03)
161 obs / 178.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.34 (0.941.85)
00.351.4
Missense OE0.90 (0.791.03)
00.61.4
Synonymous OE1.04
01.21.6
LoF obs/exp: 20 / 14.9Missense obs/exp: 161 / 178.3Syn Z: -0.28

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PEX12 · 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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