7-Dehydrocholesterol-dependent proteolysis of HMG-CoA reductase suppresses sterol biosynthesis in a mouse model of Smith-Lemli-Opitz/RSH syndrome

Journal of Clinical Investigation

Volumn 108, Issue 6, 2001, Pages 905-915

  Fitzky, B U ^a   Moebius, F F ^a   Asaoka, H ^a   Waage Baudet, H ^a   Xu, L ^a   Xu, G ^a   Maeda, N ^a   Kluckman, K ^a   Hiller, S ^a   Yu, H ^a   Batta, A K ^a   Shefer, S ^a   Chen, T ^a   Salen, G ^a   Sulik, K ^a   Simoni, R D ^a   Ness, G C ^a   Glossmann, H ^a   Patel, S B ^a   Tint, G S ^a  

^a VETERANS AFFAIRS MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

7 DEHYDROCHOLESTEROL; CHOLESTEROL; HYDROXYMETHYLGLUTARYL COENZYME A REDUCTASE; LOW DENSITY LIPOPROTEIN RECEPTOR; MESSENGER RNA;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BLADDER MALFORMATION; CLEFT PALATE; CONTROLLED STUDY; DISEASE EXACERBATION; ENZYME ACTIVITY; ENZYME MECHANISM; ENZYME REPRESSION; GENE MUTATION; LETHALITY; LUNG MALFORMATION; MOUSE; NEWBORN; NONHUMAN; PRIORITY JOURNAL; PROTEIN DEGRADATION; SMITH LEMLI OPITZ SYNDROME; STEROL SYNTHESIS;

ANIMALS; ANIMALS, NEWBORN; DEHYDROCHOLESTEROLS; DISEASE MODELS, ANIMAL; DNA-BINDING PROTEINS; GENE TARGETING; HUMANS; HYDROXYMETHYLGLUTARYL COA REDUCTASES; MICE; MICE, KNOCKOUT; OXIDOREDUCTASES; OXIDOREDUCTASES ACTING ON CH-CH GROUP DONORS; RECEPTORS, LDL; RNA, MESSENGER; SMITH-LEMLI-OPITZ SYNDROME; STEROL REGULATORY ELEMENT BINDING PROTEIN 2; STEROLS; TRANSCRIPTION FACTORS;

EID: 0034829757     PISSN: 00219738     EISSN: None     Source Type: Journal    
DOI: 10.1172/JCI200112103     Document Type: Article

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