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Volumn 106, Issue 7, 2009, Pages 2093-2094

Unraveling the sterol-trafficking defect in Niemann-Pick C disease

Author keywords

[No Author keywords available]

Indexed keywords

CHOLESTEROL; CYCLODEXTRIN; STEROL;

EID: 60549095412     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.0812934106     Document Type: Note
Times cited : (20)

References (10)
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    • Purified NPC1 protein. I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein
    • Infante RE, et al. (2008) Purified NPC1 protein. I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein. J Biol Chem 283:1052-1063.
    • (2008) J Biol Chem , vol.283 , pp. 1052-1063
    • Infante, R.E.1
  • 3
    • 33845994402 scopus 로고    scopus 로고
    • NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols
    • Liou HL, et al. (2006) NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols. J Biol Chem 281:36710-36723.
    • (2006) J Biol Chem , vol.281 , pp. 36710-36723
    • Liou, H.L.1
  • 4
    • 55749083068 scopus 로고    scopus 로고
    • NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes
    • Infante RE, et al. (2008) NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes. Proc Natl Acad Sci USA 105:15287-15292.
    • (2008) Proc Natl Acad Sci USA , vol.105 , pp. 15287-15292
    • Infante, R.E.1
  • 5
    • 11144355005 scopus 로고    scopus 로고
    • Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport
    • Sleat DE, et al. (2004) Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport. Proc Natl Acad Sci USA 101:5886-5891.
    • (2004) Proc Natl Acad Sci USA , vol.101 , pp. 5886-5891
    • Sleat, D.E.1
  • 6
    • 42949148332 scopus 로고    scopus 로고
    • Genetic variations and treatments that affect the lifespan of the NPC1 mouse
    • Liu B, Li H, Repa JJ, Turley SD, Dietschy JM (2008) Genetic variations and treatments that affect the lifespan of the NPC1 mouse. J Lipid Res 49:663-669.
    • (2008) J Lipid Res , vol.49 , pp. 663-669
    • Liu, B.1    Li, H.2    Repa, J.J.3    Turley, S.D.4    Dietschy, J.M.5
  • 7
    • 55549134611 scopus 로고    scopus 로고
    • Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium
    • Lloyd-Evans E, et al. (2008) Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium. Nat Med 14:1247-1255.
    • (2008) Nat Med , vol.14 , pp. 1247-1255
    • Lloyd-Evans, E.1
  • 8
    • 34147137671 scopus 로고    scopus 로고
    • Lysosomal unesterified cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease
    • Beltroy EP, Liu B, Dietschy JM, Turley SD (2007) Lysosomal unesterified cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease. J Lipid Res 48:869-881.
    • (2007) J Lipid Res , vol.48 , pp. 869-881
    • Beltroy, E.P.1    Liu, B.2    Dietschy, J.M.3    Turley, S.D.4
  • 9
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    • Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse
    • Repa JJ, et al. (2007) Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse. J Neurosci 27:14470-14480.
    • (2007) J Neurosci , vol.27 , pp. 14470-14480
    • Repa, J.J.1
  • 10
    • 15644375176 scopus 로고    scopus 로고
    • Intracellular trafficking of the free cholesterol derived from LDL cholesteryl ester is defective in vivo in Niemann-Pick C disease: Insights on normal metabolism of HDL and LDL gained from the NP-C mutation
    • Shamburek RD, et al. (1997) Intracellular trafficking of the free cholesterol derived from LDL cholesteryl ester is defective in vivo in Niemann-Pick C disease: Insights on normal metabolism of HDL and LDL gained from the NP-C mutation. J Lipid Res 38:2422-2435.
    • (1997) J Lipid Res , vol.38 , pp. 2422-2435
    • Shamburek, R.D.1


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.