LAMP2

Chr XXLD

lysosome associated membrane protein 2

Also known as: CD107b, DND, LAMP-2, LAMPB, LGP-96, LGP110

The protein is a lysosomal membrane glycoprotein that provides carbohydrate ligands for selectins and functions in lysosomal protection, maintenance, and adhesion. Mutations cause Danon disease through an X-linked dominant inheritance pattern. The pathogenic mechanism involves gain-of-function effects affecting lysosomal integrity and cellular adhesion processes.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismXLDLOEUF 0.641 OMIM phenotype
Clinical SummaryLAMP2
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Gene-Disease Validity (ClinGen)
Danon disease · XLDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.25) despite low pLI — interpret in context.
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Clinical Trials
3 active or recruiting trials — potential therapeutic options may be available
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GeneReview available — LAMP2
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.64LOEUF
pLI 0.267
Z-score 2.42
OE 0.25 (0.110.64)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.84Z-score
OE missense 0.80 (0.690.94)
116 obs / 144.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.25 (0.110.64)
00.351.4
Missense OE0.80 (0.690.94)
00.61.4
Synonymous OE0.81
01.21.6
LoF obs/exp: 3 / 12.1Missense obs/exp: 116 / 144.4Syn Z: 1.06
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveLAMP2-related Danon diseaseLOFmonoallelic_X_heterozygous
DN
0.5771th %ile
GOF
0.6638th %ile
LOF
0.3842th %ile

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

LAMP2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 5 results · since 2015Search PubMed ↗