APLP2

Chr 11

amyloid beta precursor like protein 2

Also known as: APLP-2, APPH, APPL2, CDEBP

APLP2 encodes amyloid precursor-like protein 2, which inhibits multiple coagulation factors and proteases including trypsin, chymotrypsin, and factor XIA, and works synergistically with amyloid precursor protein to mediate neuromuscular transmission and synaptic plasticity. Mutations cause autosomal recessive developmental and epileptic encephalopathy with movement abnormalities, typically presenting in infancy with seizures, developmental delay, and neuromotor dysfunction. The gene shows significant constraint against loss-of-function variation (LOEUF 0.393), indicating intolerance to protein-truncating mutations.

Summary from RefSeq, UniProt
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0
Active trials
9
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.39
LOEUF
DN
Mechanism· predicted
Clinical SummaryAPLP2
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.23) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.39LOEUF
pLI 0.380
Z-score 4.54
OE 0.23 (0.140.39)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
-0.00Z-score
OE missense 1.00 (0.931.08)
455 obs / 454.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.23 (0.140.39)
00.351.4
Missense OE1.00 (0.931.08)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 9 / 39.9Missense obs/exp: 455 / 454.7Syn Z: 0.05
DN
0.74top 25%
GOF
0.5563th %ile
LOF
0.3744th %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

APLP2 · 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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