LIPA

Chr 10

lipase A, lysosomal acid type

Also known as: CESD, LAL

This gene encodes lipase A, the lysosomal acid lipase (also known as cholesterol ester hydrolase). This enzyme functions in the lysosome to catalyze the hydrolysis of cholesteryl esters and triglycerides. Mutations in this gene can result in Wolman disease and cholesteryl ester storage disease. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jan 2014]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtCholesteryl ester storage disease
UniProtWolman disease

Clinical highlights

Gene-disease validity (ClinGen)
lysosomal acid lipase deficiency · ARDefinitivesufficient evidence for diagnostic panels
4
Active trials
166
Pubs (1 yr)
P/LP submissions
P/LP missense
1.00
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — LIPA
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.00LOEUF
pLI 0.000
Z-score 1.57
OE 0.63 (0.411.00)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.23Z-score
OE missense 0.96 (0.851.07)
205 obs / 214.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.63 (0.411.00)
00.351.4
Missense OE?0.96 (0.851.07)
00.61.4
Synonymous OE?1.20
01.21.6
LoF obs/exp: 13 / 20.7Missense obs/exp: 205 / 214.4Syn Z: -1.38

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

LIPA · protein map & ClinVar variants

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