209 related articles for article (PubMed ID: 29246491)
1. Impact of loss of SOAT2 function on disease progression in the lysosomal acid lipase-deficient mouse.
Lopez AM; Chuang JC; Turley SD
Steroids; 2018 Feb; 130():7-14. PubMed ID: 29246491
[TBL] [Abstract][Full Text] [Related]
2. Deletion of sterol O-acyltransferase 2 (SOAT2) function in mice deficient in lysosomal acid lipase (LAL) dramatically reduces esterified cholesterol sequestration in the small intestine and liver.
Lopez AM; Posey KS; Turley SD
Biochem Biophys Res Commun; 2014 Nov; 454(1):162-6. PubMed ID: 25450374
[TBL] [Abstract][Full Text] [Related]
3. PRD125, a potent and selective inhibitor of sterol O-acyltransferase 2 markedly reduces hepatic cholesteryl ester accumulation and improves liver function in lysosomal acid lipase-deficient mice.
Lopez AM; Chuang JC; Posey KS; Ohshiro T; Tomoda H; Rudel LL; Turley SD
J Pharmacol Exp Ther; 2015 Nov; 355(2):159-67. PubMed ID: 26283692
[TBL] [Abstract][Full Text] [Related]
4. Niemann-Pick C1-deficient mice lacking sterol O-acyltransferase 2 have less hepatic cholesterol entrapment and improved liver function.
Lopez AM; Jones RD; Repa JJ; Turley SD
Am J Physiol Gastrointest Liver Physiol; 2018 Oct; 315(4):G454-G463. PubMed ID: 29878847
[TBL] [Abstract][Full Text] [Related]
5. Hepatic entrapment of esterified cholesterol drives continual expansion of whole body sterol pool in lysosomal acid lipase-deficient mice.
Aqul A; Lopez AM; Posey KS; Taylor AM; Repa JJ; Burns DK; Turley SD
Am J Physiol Gastrointest Liver Physiol; 2014 Oct; 307(8):G836-47. PubMed ID: 25147230
[TBL] [Abstract][Full Text] [Related]
6. Ezetimibe markedly attenuates hepatic cholesterol accumulation and improves liver function in the lysosomal acid lipase-deficient mouse, a model for cholesteryl ester storage disease.
Chuang JC; Lopez AM; Posey KS; Turley SD
Biochem Biophys Res Commun; 2014 Jan; 443(3):1073-7. PubMed ID: 24370824
[TBL] [Abstract][Full Text] [Related]
7. Molecular markers of brain cholesterol homeostasis are unchanged despite a smaller brain mass in a mouse model of cholesteryl ester storage disease.
Aqul AA; Ramirez CM; Lopez AM; Burns DK; Repa JJ; Turley SD
Lipids; 2022 Jan; 57(1):3-16. PubMed ID: 34618372
[TBL] [Abstract][Full Text] [Related]
8. Quantitation of the rates of hepatic and intestinal cholesterol synthesis in lysosomal acid lipase-deficient mice before and during treatment with ezetimibe.
Chuang JC; Lopez AM; Turley SD
Biochem Pharmacol; 2017 Jul; 135():116-125. PubMed ID: 28322747
[TBL] [Abstract][Full Text] [Related]
9. Delineation of metabolic responses of Npc1
Ramirez CM; Taylor AM; Lopez AM; Repa JJ; Turley SD
Steroids; 2020 Dec; 164():108725. PubMed ID: 32890578
[TBL] [Abstract][Full Text] [Related]
10. Ontogenesis and Modulation of Intestinal Unesterified Cholesterol Sequestration in a Mouse Model of Niemann-Pick C1 Disease.
Lopez AM; Ramirez CM; Taylor AM; Jones RD; Repa JJ; Turley SD
Dig Dis Sci; 2020 Jan; 65(1):158-167. PubMed ID: 31312996
[TBL] [Abstract][Full Text] [Related]
11. Expression and functional characterization of human lysosomal acid lipase gene (LIPA) mutation responsible for cholesteryl ester storage disease (CESD) phenotype.
Rajamohan F; Reyes AR; Ruangsiriluk W; Hoth LR; Han S; Caspers N; Tu M; Ward J; Kurumbail RG
Protein Expr Purif; 2015 Jun; 110():22-9. PubMed ID: 25620107
[TBL] [Abstract][Full Text] [Related]
12. Lysosomal acid lipase deficiency: correction of lipid storage by adenovirus-mediated gene transfer in mice.
Du H; Heur M; Witte DP; Ameis D; Grabowski GA
Hum Gene Ther; 2002 Jul; 13(11):1361-72. PubMed ID: 12162818
[TBL] [Abstract][Full Text] [Related]
13. Hepatic cholesteryl ester accumulation in lysosomal acid lipase deficiency: non-invasive identification and treatment monitoring by magnetic resonance.
Thelwall PE; Smith FE; Leavitt MC; Canty D; Hu W; Hollingsworth KG; Thoma C; Trenell MI; Taylor R; Rutkowski JV; Blamire AM; Quinn AG
J Hepatol; 2013 Sep; 59(3):543-9. PubMed ID: 23624251
[TBL] [Abstract][Full Text] [Related]
14. Lysosomal acid lipase deficiency: diagnosis and treatment of Wolman and Cholesteryl Ester Storage Diseases.
Porto AF
Pediatr Endocrinol Rev; 2014 Sep; 12 Suppl 1():125-32. PubMed ID: 25345094
[TBL] [Abstract][Full Text] [Related]
15. Managing Cardiovascular Risk in Lysosomal Acid Lipase Deficiency.
Maciejko JJ
Am J Cardiovasc Drugs; 2017 Jun; 17(3):217-231. PubMed ID: 28197978
[TBL] [Abstract][Full Text] [Related]
16. Acute sterol o-acyltransferase 2 (SOAT2) knockdown rapidly mobilizes hepatic cholesterol for fecal excretion.
Marshall SM; Gromovsky AD; Kelley KL; Davis MA; Wilson MD; Lee RG; Crooke RM; Graham MJ; Rudel LL; Brown JM; Temel RE
PLoS One; 2014; 9(6):e98953. PubMed ID: 24901470
[TBL] [Abstract][Full Text] [Related]
17. Hepatocyte-specific deletion of lysosomal acid lipase leads to cholesteryl ester but not triglyceride or retinyl ester accumulation.
Pajed L; Wagner C; Taschler U; Schreiber R; Kolleritsch S; Fawzy N; Pototschnig I; Schoiswohl G; Pusch LM; Wieser BI; Vesely P; Hoefler G; Eichmann TO; Zimmermann R; Lass A
J Biol Chem; 2019 Jun; 294(23):9118-9133. PubMed ID: 31023823
[TBL] [Abstract][Full Text] [Related]
18. Extended use of a selective inhibitor of acid lipase for the diagnosis of Wolman disease and cholesteryl ester storage disease.
Civallero G; De Mari J; Bittar C; Burin M; Giugliani R
Gene; 2014 Apr; 539(1):154-6. PubMed ID: 24508470
[TBL] [Abstract][Full Text] [Related]
19. Reversal of advanced disease in lysosomal acid lipase deficient mice: a model for lysosomal acid lipase deficiency disease.
Sun Y; Xu YH; Du H; Quinn B; Liou B; Stanton L; Inskeep V; Ran H; Jakubowitz P; Grilliot N; Grabowski GA
Mol Genet Metab; 2014 Jul; 112(3):229-41. PubMed ID: 24837159
[TBL] [Abstract][Full Text] [Related]
20. Molecular and clinical characterization of a series of patients with childhood-onset lysosomal acid lipase deficiency. Retrospective investigations, follow-up and detection of two novel LIPA pathogenic variants.
Pisciotta L; Tozzi G; Travaglini L; Taurisano R; Lucchi T; Indolfi G; Papadia F; Di Rocco M; D'Antiga L; Crock P; Vora K; Nightingale S; Michelakakis H; Garoufi A; Lykopoulou L; Bertolini S; Calandra S
Atherosclerosis; 2017 Oct; 265():124-132. PubMed ID: 28881270
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
