LAMP1

Chr 13

lysosome associated membrane protein 1

Also known as: CD107a, LAMPA, LGP120

LAMP1 encodes a lysosomal membrane glycoprotein that regulates lysosomal pH by inhibiting the proton channel TMEM175, facilitates autophagy and cholesterol homeostasis, and plays a critical role in NK cell cytotoxicity through cytotoxic granule trafficking. Mutations cause autosomal recessive lysosomal storage disorders with neurodegeneration and immune dysfunction. The gene is highly constrained (pLI 0.87, LOEUF 0.40), indicating intolerance to loss-of-function variants.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
220
Pubs (1 yr)
19
P/LP submissions
0%
P/LP missense
0.40
LOEUF
Mechanism
Clinical SummaryLAMP1
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.87) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
19 unique Pathogenic / Likely Pathogenic· 62 VUS of 100 total submissions
📖
GeneReview available — LAMP1
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.40LOEUF
pLI 0.875
Z-score 3.19
OE 0.13 (0.050.40)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.83Z-score
OE missense 0.85 (0.770.96)
220 obs / 257.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.13 (0.050.40)
00.351.4
Missense OE0.85 (0.770.96)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 2 / 15.6Missense obs/exp: 220 / 257.4Syn Z: -0.50

ClinVar Variant Classifications

100 submitted variants in ClinVar

Classification Summary

Pathogenic19
VUS62
Likely Benign7
19
Pathogenic
62
VUS
7
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
19
0
19
Likely Pathogenic
0
0
0
0
0
VUS
0
58
4
0
62
Likely Benign
0
3
2
2
7
Benign
0
0
0
0
0
Total06125288

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

LAMP1 · 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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