Hearing Loss and Hair Cell Death in Mice Given the Cholesterol-Chelating Agent Hydroxypropyl-β-Cyclodextrin

PLoS ONE

Volumn 7, Issue 12, 2012, Pages

  Crumling, Mark A ^a   Liu, Liqian ^a   Thomas, Paul V ^a   Benson, Jennifer ^a   Kanicki, Ariane ^a   Kabara, Lisa ^a   Hälsey, Karin ^a   Dolan, David ^a   Duncan, R Keith ^a  

^a UNIVERSITY OF MICHIGAN HEALTH SYSTEM   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

2 HYDROXYPROPYL BETA CYCLODEXTRIN; PRESTIN;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTICLE; AUDITORY THRESHOLD; CELL DEATH; CELL MEMBRANE; CELL SURVIVAL; COCHLEA; CONTROLLED STUDY; CORRELATION ANALYSIS; DISEASE SEVERITY; DRUG DOSE COMPARISON; EVOKED AUDITORY RESPONSE; HAIR CELL; HEARING IMPAIRMENT; HISTOPATHOLOGY; MOUSE; NONHUMAN; PROTEIN LOCALIZATION; REPEATED DRUG DOSE;

ANIMALS; ANTICHOLESTEREMIC AGENTS; BETA-CYCLODEXTRINS; CELL DEATH; DOSE-RESPONSE RELATIONSHIP, DRUG; DRUG ADMINISTRATION SCHEDULE; DRUG EVALUATION, PRECLINICAL; HAIR CELLS, AUDITORY; HEARING LOSS; INFUSIONS, PARENTERAL; MICE;

MUS;

EID: 84871681568     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0053280     Document Type: Article

References (30)

1. Maxfield FR, van Meer G, (2010) Cholesterol, the central lipid of mammalian cells. Curr Opin Cell Biol 22: 422-429.
2. Gimpl G, (2010) Cholesterol-protein interaction: methods and cholesterol reporter molecules. Subcell Biochem 51: 1-45.
3. Di Berardino F, Alpini D, Ambrosetti U, Amadeo C, Cesarani A, (2007) Sensorineural hearing-loss in the Smith-Lemli-Opitz syndrome. International Journal of Pediatric Otorhinolaryngology Extra 2: 169-172.
4. Yanjanin NM, Velez JI, Gropman A, King K, Bianconi SE, et al. (2010) Linear clinical progression, independent of age of onset, in Niemann-Pick disease, type C. Am J Med Genet B Neuropsychiatr Genet. 153B: 132-140.
5. Levic S, Yamoah EN, (2011) Plasticity in membrane cholesterol contributes toward electrical maturation of hearing. J Biol Chem 286: 5768-5773.
6. Purcell EK, Liu L, Thomas PV, Duncan RK, (2011) Cholesterol influences voltage-gated calcium channels and BK-type potassium channels in auditory hair cells. PLoS One 6: e26289.
7. Rajagopalan L, Greeson JN, Xia A, Liu H, Sturm A, et al. (2007) Tuning of the outer hair cell motor by membrane cholesterol. J Biol Chem 282: 36659-36670.
8. Davidson CD, Ali NF, Micsenyi MC, Stephney G, Renault S, et al. (2009) Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression. PLoS One 4: e6951.
9. Liu B, Ramirez CM, Miller AM, Repa JJ, Turley SD, et al. (2010) Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid. J Lipid Res 51: 933-944.
10. Loftsson T, Brewster ME, (2010) Pharmaceutical applications of cyclodextrins: basic science and product development. J Pharm Pharmacol 62: 1607-1621.
11. Ward S, O'Donnell P, Fernandez S, Vite CH, (2010) 2-hydroxypropyl-beta-cyclodextrin raises hearing threshold in normal cats and in cats with Niemann-Pick type C disease. Pediatr Res 68: 52-56.
12. Zheng QY, Johnson KR, Erway LC, (1999) Assessment of hearing in 80 inbred strains of mice by ABR threshold analyses. Hear Res 130: 94-107.
13. Muralidhar A, Borbon IA, Esharif DM, Ke W, Manacheril R, et al. (2011) Pulmonary function and pathology in hydroxypropyl-beta-cyclodextin-treated and untreated Npc1(-)/(-) mice. Mol Genet Metab 103: 142-147.
14. Ramirez CM, Liu B, Taylor AM, Repa JJ, Burns DK, et al. (2010) Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life. Pediatr Res 68: 309-315.
15. Karolyi IJ, Dootz GA, Halsey K, Beyer L, Probst FJ, et al. (2007) Dietary thyroid hormone replacement ameliorates hearing deficits in hypothyroid mice. Mamm Genome 18: 596-608.
16. Mhatre AN, Tajudeen B, Welt EM, Wartmann C, Long GR, et al. (2010) Temporary reduction of distortion product otoacoustic emissions (DPOAEs) immediately following auditory brainstem response (ABR). Hear Res 269: 180-185.
17. Muller M, von Hunerbein K, Hoidis S, Smolders JW, (2005) A physiological place-frequency map of the cochlea in the CBA/J mouse. Hear Res 202: 63-73.
18. Gould S, Scott RC, (2005) 2-Hydroxypropyl-beta-cyclodextrin (HP-beta-CD): a toxicology review. Food Chem Toxicol 43: 1451-1459.
19. Ashmore J, (2008) Cochlear outer hair cell motility. Physiol Rev 88: 173-210.
20. Cheatham MA, Huynh KH, Gao J, Zuo J, Dallos P, (2004) Cochlear function in Prestin knockout mice. J Physiol 560: 821-830.
21. Wu X, Gao J, Guo Y, Zuo J, (2004) Hearing threshold elevation precedes hair-cell loss in prestin knockout mice. Brain Res Mol Brain Res 126: 30-37.
22. Brownell WE, Jacob S, Hakizimana P, Ulfendahl M, Fridberger A, (2011) Membrane cholesterol modulates cochlear electromechanics. Pflugers Arch 461: 677-686.
23. Zheng J, Long KB, Matsuda KB, Madison LD, Ryan AD, et al. (2003) Genomic characterization and expression of mouse prestin, the motor protein of outer hair cells. Mamm Genome 14: 87-96.
24. Kalinec F, Holley MC, Iwasa KH, Lim DJ, Kachar B, (1992) A membrane-based force generation mechanism in auditory sensory cells. Proc Natl Acad Sci U S A 89: 8671-8675.
25. Santos-Sacchi J, Kakehata S, Kikuchi T, Katori Y, Takasaka T, (1998) Density of motility-related charge in the outer hair cell of the guinea pig is inversely related to best frequency. Neurosci Lett 256: 155-158.
26. Nguyen TV, Brownell WE, (1998) Contribution of membrane cholesterol to outer hair cell lateral wall stiffness. Otolaryngol Head Neck Surg 119: 14-20.
27. Levitan I, Fang Y, Rosenhouse-Dantsker A, Romanenko V, (2010) Cholesterol and ion channels. Subcell Biochem 51: 509-549.
28. Kharkovets T, Dedek K, Maier H, Schweizer M, Khimich D, et al. (2006) Mice with altered KCNQ4 K+ channels implicate sensory outer hair cells in human progressive deafness. EMBO J 25: 642-652.
29. Ohtani Y, Irie T, Uekama K, Fukunaga K, Pitha J, (1989) Differential effects of alpha-, beta- and gamma-cyclodextrins on human erythrocytes. Eur J Biochem 186: 17-22.
30. Oesterle EC, Campbell S, Taylor RR, Forge A, Hume CR, (2008) Sox2 and JAGGED1 expression in normal and drug-damaged adult mouse inner ear. J Assoc Res Otolaryngol 9: 65-89.