GABAA receptor expression and inhibitory post-synaptic currents in cerebellar Purkinje cells in dystrophin-deficient mdx mice

Clinical and Experimental Pharmacology and Physiology

Volumn 35, Issue 2, 2008, Pages 207-210

  Kueh, S L L ^a   Head, S I ^a   Morley, J W ^a,b  

^a UNIVERSITY OF NEW SOUTH WALES   (Australia)

^b UNIVERSITY OF WESTERN SYDNEY   (Australia)

Author keywords

Cerebellum; Duchenne muscular dystrophy; Dystrophinopathy; Inhibition; Purkinje

Indexed keywords

4 AMINOBUTYRIC ACID A RECEPTOR; DYSTROPHIN;

AMPLITUDE MODULATION; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BEHAVIOR DISORDER; COGNITION; CONTROLLED STUDY; IMMUNOREACTIVITY; MALE; MOUSE; NONHUMAN; POSTSYNAPTIC MEMBRANE; PROTEIN EXPRESSION; PURKINJE CELL;

ANIMALS; BEHAVIOR, ANIMAL; BLOTTING, WESTERN; DISEASE MODELS, ANIMAL; INHIBITORY POSTSYNAPTIC POTENTIALS; KINETICS; MICE; MICE, INBRED MDX; MUSCULAR DYSTROPHY, DUCHENNE; PATCH-CLAMP TECHNIQUES; PURKINJE CELLS; RECEPTORS, GABA-A; SYNAPTIC MEMBRANES;

EID: 38149137177     PISSN: 03051870     EISSN: 14401681     Source Type: Journal    
DOI: 10.1111/j.1440-1681.2007.04816.x     Document Type: Article

References (29)

1. Emery AEH. Duchenne Muscular Dystrophy. Oxford Unversity Press, New York. 1987.
2. Emery AEH. Population frequencies of inherited neuromuscular diseases: A world survey. Neuromuscul. Disord. 1991 1: 19 29.
3. Lidov HGW, Byers TJ, Watkins SC et al. Localization of dystrophin to postsynaptic regions of central nervous system cortical neurons. Nature 1990 348: 725 58.
4. Lidov HG, Byers TJ, Kunkel LM. The distribution of dystrophin in the murine central nervous system: An immunocytochemical study. Neuroscience 1993 54: 167 87.
5. Uchino M, Teramoto H, Naoe H et al. Localisation and characterisation of dystrophin in the central nervous system of controls and patients with Duchenne muscular dystrophy. J. Neurol. Neurosurg. Psychiatry 1994 57: 426 9.
6. Uchino M, Yoshioka K, Miike T et al. Dystrophin and dystrophin-related protein in the brains of normal and mdx mice. Muscle Nerve 1994 17: 533 8.
7. Bresolin N, Castelli E, Comi GP et al. Cognitive impairment in Duchenne muscular dystrophy. Neuromuscul. Disord. 1994 4: 359 69.
8. Leibowitz D, Dubowitz V. Intellect and behaviour in Duchenne muscular dystrophy. Dev. Med. Child. Neurol. 1981 23: 577 90.
9. Anderson JL, Head SI, Rae C et al. Brain function in Duchenne muscular dystrophy. Brain 2002 125: 4 13.
10. A receptors in mice lacking dystrophin (mdx mice). Eur. J. Neurosci. 1999 11: 4457 62.
11. Kong J, Anderson JE. Dystrophin is required for organizing large acetylcholine receptor aggregates. Brain Res. 1999 839: 298 304.
12. Connors NC, Kofuji P. Dystrophin Dp71 is critical for the clustered localization of potassium channels in retinal glial cells. J. Neurosci. 2002 22: 4321 7.
13. Vajda Z, Pedersen M, Fuchtbauer E-M et al. Delayed onset of brain edema and mislocalization of aquaporin-4 in dystrophin-null transgenic mice. Proc. Natl Acad. Sci. USA 2002 99: 13 131 6.
14. Grady RM, Wozniak DF, Ohlemiller KK et al. Cerebellar synaptic defects and abnormal motor behavior in mice lacking alpha- and beta-dystrobrevin. J. Neurosci. 2006 26: 2841 51.
15. Levi S, Grady RM, Henry MD et al. Dystroglycan is selectively associated with Inhibitory GABAergic synapses but is dispensable for their differentiation. J. Neurosci. 2002 22: 4274 85.
16. Areceptors). Biochem. Soc. Trans. 2003 31: 889 92.
17. Muntoni F, Mateddu A, Serra G. Passive avoidance deficit in the mdx mouse. Neuromuscul. Disord. 1991 1: 121 3.
18. mdx mouse. Neurobiol. Dis. 2004 17: 10 20.
19. Cui C-H, Uyama T, Miyado K et al. Menstrual blood-derived cells confer human dystrophin expression in the murine model of Duchenne muscular dystrophy via cell fusion and myogenic transdifferentiation. Mol. Biol. Cell 2007 18: 1586 94.
20. A receptors in the cerebellum: Effects of targeted subunit gene deletions. Cerebellum 2006 5: 275 85.
21. Kueh SLL, Head SI, Morley JW. A colony of mdx dystrophic mice with genetically identical littermate controls. Proc. Aust. Physiol. Soc. 2004 35: 99 (Abstract).
22. A receptor number underlie variation in GABA mini amplitude. Neuron 1997 19: 697 709.
23. + channel blockers. Br. J. Pharmacol. 1998 125: 1375 81.
24. Wallis T, Bubb WA, McQuillan JA et al. For want of a nail. Ramifications of a single gene deletion, dystrophin, in the brain of the mouse. Front. Biosci. 2004 9: 74 84.
25. A receptors and gephyrin. J. Neurosci. 2002 22: 4805 13.
26. Anderson JL, Head SI, Morley JW. Altered inhibitory input to purkinje cells of dystrophin-deficient mice. Brain Res. 2003 982: 280 3.
27. A receptor activation and intracellular calcium elevation. Neuron 2004 44: 701 13.
28. Xie Z, Currie KPM, Fox AP. Etomidate elevates intracellular calcium levels and promotes catecholamine secretion in bovine chromaffin cells. J. Physiol. 2004 560: 677 90.
29. Anderson JL, Head SI, Morley JW. Long term depression is reduced in cerebellar Purkinje cells of dystrophin-deficient mdx mice. Brain Res. 2004 1019: 289 92.