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Volumn 58, Issue 3, 2013, Pages 850-852

Therapy for lysosomal acid lipase deficiency: Replacing a missing link

Author keywords

[No Author keywords available]

Indexed keywords

ACID LIPASE; AMINO ACID; CHOLESTEROL; CHOLESTEROL ESTER; FATTY ACID; FERRITIN; LOW DENSITY LIPOPROTEIN RECEPTOR; LYSOSOMAL ACID LIPASE; OLIGOSACCHARIDE; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR GAMMA; STEROL REGULATORY ELEMENT BINDING PROTEIN; UNCLASSIFIED DRUG;

EID: 84883225499     PISSN: 02709139     EISSN: 15273350     Source Type: Journal    
DOI: 10.1002/hep.26366     Document Type: Article
Times cited : (16)

References (9)
  • 1
    • 0021040946 scopus 로고
    • Lysosomes revisited
    • deDuve, C. Lysosomes revisited. Eur J Biochem 1983;137:391.
    • (1983) Eur J Biochem , vol.137 , pp. 391
    • deDuve, C.1
  • 2
    • 0036251153 scopus 로고    scopus 로고
    • SREBPs: activators of the complete program of cholesterol and fatty acid synthesis in the liver
    • Horton JD, Goldstein JL, Brown MS. SREBPs: activators of the complete program of cholesterol and fatty acid synthesis in the liver. J Clin Invest 2002;109:1125-1131.
    • (2002) J Clin Invest , vol.109 , pp. 1125-1131
    • Horton, J.D.1    Goldstein, J.L.2    Brown, M.S.3
  • 4
    • 0016836751 scopus 로고
    • Role of lysosomal acid lipase in the metabolism of plasma low density lipoprotein
    • Goldstein JL, Dana SE, Faust JR, Beaudet AL, Brown ML. Role of lysosomal acid lipase in the metabolism of plasma low density lipoprotein. J Biol Chem 1975;250:8487-8795.
    • (1975) J Biol Chem , vol.250 , pp. 8487-8795
    • Goldstein, J.L.1    Dana, S.E.2    Faust, J.R.3    Beaudet, A.L.4    Brown, M.L.5
  • 5
    • 84902540473 scopus 로고    scopus 로고
    • Lysosomal acid lipase deficiencies
    • Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, eds), 9th ed. New York: McGraw-Hill
    • Grabowski GA, Charnas LW, Du H. Lysosomal acid lipase deficiencies. In: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, et al. (eds). Metabolic and Molecular Bases of Inherited Disease, 9th ed. New York: McGraw-Hill; 2012.
    • (2012) Metabolic and Molecular Bases of Inherited Disease
    • Grabowski, G.A.1    Charnas, L.W.2    Du, H.3
  • 7
    • 84883460510 scopus 로고    scopus 로고
    • Clinical effect and safety profile of recombinant human lysosomal acid lipase in patients with cholesteryl ester storage disease
    • Balwani M, Breen C, Enns GM, Deegan PB, Honzík T, Jones S, et al. Clinical effect and safety profile of recombinant human lysosomal acid lipase in patients with cholesteryl ester storage disease. Hepatology 2013; 58:950-957.
    • (2013) Hepatology , vol.58 , pp. 950-957
    • Balwani, M.1    Breen, C.2    Enns, G.M.3    Deegan, P.B.4    Honzík, T.5    Jones, S.6
  • 8
    • 0031657974 scopus 로고    scopus 로고
    • Targeted disruption of the mouse lysosomal acid lipase gene: a cholesteryl ester storage disease clinical phenotype and Wolman disease biochemistry
    • Du H, Dumanu M, Witte D, Grabowski GA. Targeted disruption of the mouse lysosomal acid lipase gene: a cholesteryl ester storage disease clinical phenotype and Wolman disease biochemistry. Hum Mol Genet 1998;7:1347-1354.
    • (1998) Hum Mol Genet , vol.7 , pp. 1347-1354
    • Du, H.1    Dumanu, M.2    Witte, D.3    Grabowski, G.A.4
  • 9
    • 28144437047 scopus 로고    scopus 로고
    • The role of mannosylated enzyme and the mannose receptor in enzyme replacement therapy
    • Du H, Levine M, Ganesa C, Witte DP, Cole ES, Grabowski GA. The role of mannosylated enzyme and the mannose receptor in enzyme replacement therapy. Am J Hum Genet 2005;77:1061-1074.
    • (2005) Am J Hum Genet , vol.77 , pp. 1061-1074
    • Du, H.1    Levine, M.2    Ganesa, C.3    Witte, D.P.4    Cole, E.S.5    Grabowski, G.A.6


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.