Pregnane X receptor (PXR) activation: A mechanism for neuroprotection in a mouse model of Niemann-Pick C disease

Proceedings of the National Academy of Sciences of the United States of America

Volumn 103, Issue 37, 2006, Pages 13807-13812

  Langmade, S Joshua ^a   Gale, Sarah E ^a   Frolov, Andrey ^a   Mohri, Ikuko ^b   Suzuki, Kinuko ^b   Mellon, Synthia H ^c   Walkley, Steven U ^d   Covey, Douglas F ^a   Schaffer, Jean E ^a   Ory, Daniel S ^a  

^a WASHINGTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b UNIVERSITY OF NORTH CAROLINA SCHOOL OF MEDICINE   (United States)

^c UNIVERSITY OF CALIFORNIA   (United States)

^d ALBERT EINSTEIN COLLEGE OF MEDICINE   (United States)

Author keywords

Allopregnanolone; Cholesterol; Neurodegeneration; Neurosteroid

Indexed keywords

3ALPHA HYDROXY 5ALPHA PREGNAN 20 ONE; 4 AMINOBUTYRIC ACID A RECEPTOR; N (2,2,2 TRIFLUOROETHYL) N [4 [2,2,2 TRIFLUORO 1 HYDROXY 1 (TRIFLUOROMETHOXY)ETHYL]PHENYL]BENZENESULFONAMIDE; NEUROSTEROID; OXYSTEROL; PREGNANE X RECEPTOR;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CHOLESTEROL TRANSPORT; CONTROLLED STUDY; CORRELATION ANALYSIS; DRUG EFFECT; GENE; LIFESPAN; MICROGLIA; MOUSE; NERVE DEGENERATION; NEUROPROTECTION; NIEMANN PICK DISEASE; NONHUMAN; NPC1 GENE; PRIORITY JOURNAL; PURKINJE CELL; STEREOISOMERISM;

ANIMALS; CEREBELLUM; DISEASE MODELS, ANIMAL; GENE EXPRESSION; MICE; MICE, KNOCKOUT; MICROGLIA; MUTATION; NEUROPROTECTIVE AGENTS; NIEMANN-PICK DISEASES; PREGNANOLONE; PROTEINS; PURKINJE CELLS; RECEPTORS, GABA-A; RECEPTORS, STEROID; STEREOISOMERISM; SULFONAMIDES;

EID: 33748793667     PISSN: 00278424     EISSN: None     Source Type: Journal    
DOI: 10.1073/pnas.0606218103     Document Type: Article

References (31)

1. Walkley SU, Suzuki K (2004) Biochim Biophys Acta 1685:48-62.
2. Neufeld EB, Cooney AM, Pitha J, Dawidowicz EA, Dwyer NK, Pentchev PG, Blanchette-Mackie EJ (1996) J Biol Chem 271:21604-21613.
3. Millard EE, Srivastava K, Traub L, Schaffer JE, Ory DS (2000) J Biol Chem 275:38445-38451.
4. Wojtanik KM, Liscum L (2003) J Biol Chem 278:14850-14856.
5. Liscum L, Faust JR (1987) J Biol Chem 262:17002-17008.
6. Pentchev PG, Comly ME, Kruth HS, Vanier MT, Wenger DA, Patel S, Brady RO (1985) Proc Natl Acad Sci USA 82:8247-8251.
7. Choi HY, Karten B, Chan T, Vance JE, Greer WL, Heidenreich RA, Garver WS, Francis GA (2003) J Biol Chem 278:32569-32577.
8. Higashi Y, Murayama S, Pentchev PG, Suzuki K (1993) Acta Neuropathol 85:175-184.
9. Loftus SK, Morris JA, Carstea ED, Gu JZ, Cummings C, Brown A, Ellison J, Ohno K, Rosenfeld MA, Tagle DA, et al. (1997) Science 277:232-235.
10. Reid P, Sakahita N, Sugii S, Ohno-Iwashita Y, Shimada Y, Hickey W, Chang T (2004) J Lipid Res 45:582-591.
11. Wu Y-P, Mizukami H, Matsuda J, Saito Y, Proia R, Suzuki K (2005) Mol Genet Metab 84:9-17.
12. Ko D, Milenkovic L, Beier S, Manuel H, Buchanan J, Scott M (2005) PLoS Genet 1:81-95.
13. Frolov A, Zielinski SE, Crowley JR, Dudley-Rucker N, Schaffer JE, Ory DS (2003) J Biol Chem 278:25517-25525.
14. Griffin L, Gong W, Verot L, Mellon S (2004) Nat Med 10:704-711.
15. Janowski BA, Willy PJ, Devi TR, Falck JR, Manglesdorf DJ (1996) Nature 383:728-731.
16. Fu X, Menke JG, Chen Y, Zhou G, MacNaul KL, Wright SD, Sparrow CP, Lund EG (2001) J Biol Chem 276:38378-38387.
17. Whitney KD, Watson MA, Collins JL, Benson WG, Stone TM, Numerick MJ, Tippin TK, Wilson JG, Winegar DA, Kliewer SA (2002) Mol Endocrinol 16:1378-1385.
18. Wittmer L, Hu Y, Kalbrenner M, Evers A, Zorumski C, Covey D (1996) Mol Pharmacol 50:1581-1586.
19. Hu Y, Wittmer L, Kalkbrenner M, Evers A, Zorumski C, Covey D (1997) J Chem Soc Perkin Trans 1 3665-3671.
20. Hagemeyer C, Rosenbrock H, Ditter M, Knoth R, Volk B (2003) Neuroscience 117:521-529.
21. Lamba V, Yasuda K, Lamba J, Assem M, Davila J, Strom S, Schuetz E (2004) Toxicol Appl Pharmacol 199:251-265.
22. Goodwin B, Hodgson E, Liddle C (1999) Mol Pharmacol 56:1329-1339.
23. Ahmad I, Lope-Piedrafta S, Bi X, Hicks C, Yao Y, Yu C, Chaitkin E, Howison C, Weberg L, Trouard T, Erickson R (2005) J Neurosci Res 82:811-821.
24. Watkins R, Wisely G, Moore L, Collins J, Lambert M, Williams S, Willson T, Kliewer SA, Redinbo M (2001) Science 292:2329-2333.
25. Shenoy S, Spencer T, Mercer-Haines N, Alipour M, Gargano M, Runge-Morris M, Kocarek T (2004) Drug Metab Dispos 32:66-71.
26. Agrawal A, Shapiro B (2005) FASEB J 19:470-472.
27. Tint G, Pentchev P, Xu G, Batta A, Shiefer S, Salen G, Honda A (1998) J Inherited Metab Dis 21:853-863.
28. Chen W, Sun Y, Welch C, Gorelik A, Leventhal AR, Tabas I, Tall AR (2001) J Biol Chem 276:43564-43569.
29. Matsuda J, Kido M, Tadano-Aritomi K, Ishizuka I, Takeda E, Suzuki K, Kuroda Y (2004) Hum Mol Genet 13:2709-2723.
30. Millard E, Gale S, Dudley N, Zhang J, Schaffer J, Dry D (2005) J Biol Chem 280:28581-28590.
31. Lehmann J, McKee D, Watson M, Willson T, Moore J, Kliewer S (1998) J Clin Invest 102:1016-1023.