Reduced lysosomal acid lipase activity: A new marker of liver disease severity across the clinical continuum of non-alcoholic fatty liver disease?
Biomarkers
/ blood
Cholesterol Esters
/ metabolism
Disease Progression
Dried Blood Spot Testing
Enzyme Replacement Therapy
/ methods
Humans
Lipid Metabolism
/ genetics
Liver
/ pathology
Liver Cirrhosis
/ pathology
Liver Function Tests
/ methods
Lysosomes
/ metabolism
Non-alcoholic Fatty Liver Disease
/ diagnosis
Severity of Illness Index
Sterol Esterase
/ blood
Triglycerides
/ metabolism
Wolman Disease
/ drug therapy
Wolman Disease
Cholesterol ester storage disease
Cirrhosis
Lysosomal acid lipase
Non-alcoholic fatty liver disease
Non-alcoholic steatohepatitis
Wolman disease
Journal
World journal of gastroenterology
ISSN: 2219-2840
Titre abrégé: World J Gastroenterol
Pays: United States
ID NLM: 100883448
Informations de publication
Date de publication:
14 Aug 2019
14 Aug 2019
Historique:
received:
08
04
2019
revised:
20
06
2019
accepted:
02
07
2019
entrez:
23
8
2019
pubmed:
23
8
2019
medline:
15
2
2020
Statut:
ppublish
Résumé
Lysosomal acid lipase (LAL) plays a key role in intracellular lipid metabolism. Reduced LAL activity promotes increased multi-organ lysosomal cholesterol ester storage, as observed in two recessive autosomal genetic diseases, Wolman disease and Cholesterol ester storage disease. Severe liver steatosis and accelerated liver fibrosis are common features in patients with genetic LAL deficiency. By contrast, few reliable data are available on the modulation of LAL activity
Identifiants
pubmed: 31435171
doi: 10.3748/wjg.v25.i30.4172
pmc: PMC6700703
doi:
Substances chimiques
Biomarkers
0
Cholesterol Esters
0
Triglycerides
0
LIPA protein, human
EC 3.1.1.13
Sterol Esterase
EC 3.1.1.13
Sebelipase alfa
K4YTU42T8G
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
4172-4180Déclaration de conflit d'intérêts
Conflict-of-interest statement: No potential conflicts of interest. No financial support.
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