Deficient mitochondrial Ca2+ buffering in the Cln8mnd mouse model of neuronal ceroid lipofuscinosis

Cell Calcium

Volumn 50, Issue 6, 2011, Pages 491-501

  Kolikova, Julia ^a   Afzalov, Ramil ^a   Surin, Alexander ^a,c   Lehesjoki, Anna Elina ^a,b   Khiroug, Leonard ^a  

^a UNIVERSITY OF HELSINKI   (Finland)

^b FOLKHÄLSAN INSTITUTE OF GENETICS   (Finland)

^c INSTITUTE OF GENERAL PATHOLOGY AND PATHOPHYSIOLOGY   (Russian Federation)

Author keywords

Excitotoxicity; Neurodegeneration; Neuronal ceroid lipofuscinosis (NCL); Patch clamp; Photolysis of caged calcium

Indexed keywords

ADENOSINE TRIPHOSPHATASE (CALCIUM); CALCIUM ION; GLUTAMIC ACID;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CALCIUM METABOLISM; CALCIUM TRANSPORT; CELL MEMBRANE; CLN8 GENE; EMBRYO; ENDOPLASMIC RETICULUM; EXCITOTOXICITY; FLUORESCENCE IMAGING; GENE; GENE MUTATION; HIPPOCAMPUS; INTERNEURON; MITOCHONDRION; MOUSE; NERVE CELL CULTURE; NEURONAL CEROID LIPOFUSCINOSIS; NONHUMAN; PATCH CLAMP; PHOTOLYSIS; PRIORITY JOURNAL; SARCOPLASMIC RETICULUM;

MUS;

EID: 82855165086     PISSN: 01434160     EISSN: 15321991     Source Type: Journal    
DOI: 10.1016/j.ceca.2011.08.004     Document Type: Article

References (41)

1. Haltia M. The neuronal ceroid-lipofuscinoses: from past to present. Biochim. Biophys. Acta 2006, 1762:850-856.
2. Siintola E., Lehesjoki A.-E., Mole S.E. Molecular genetics of the NCLs - status and perspectives. Biochim. Biophys. Acta 2006, 1762:857-864.
3. Siintola E., Topcu M., Aula N., et al. The novel neuronal ceroid lipofuscinosis gene MFSD8 encodes a putative lysosomal transporter. Am. J. Hum. Genet. 2007, 81:136-146.
4. Ranta S., Zhang Y., Ross B., et al. The neuronal ceroid lipofuscinosis in human EPMR and mnd mutant mice are associated with mutations in CLN8. Nat. Genet. 1999, 23:233-236.
5. Lonka L., Kyttälä A., Ranta S., Jalanko A., Lehesjoki A.-E. The neuronal ceroid lipofuscinosis CLN8 membrane protein is a resident of the endoplasmic reticulum. Hum. Mol. Genet. 2000, 9:1691-1697.
6. Lonka L., Salonen T., Siintola E., Kopra O., Lehesjoki A.-E., Jalanko A. Localization of wild-type and mutant neuronal ceroid lipofuscinosis CLN8 proteins in non-neuronal and neuronal cells. J. Neurosci. Res. 2004, 76:862-871.
7. Messer A., Flaherty L. Autosomal dominance in a late-onset motor neuron disease in the mouse. J. Neurogenet. 1986, 3:345-355.
8. Bronson R.T., Lake B.D., Cook S., Taylor S., Davisson M.T. Motor neuron degeneration of mice is a model of neuronal ceroid lipofuscinosis (Batten's disease). Ann. Neurol. 1993, 33:381-385.
9. Cooper J.D., Messer A., Feng A.K., Chua-Couzens J., Mobley W.C. Apparent loss and hypertrophy of interneurons in a mouse model of neuronal ceroid lipofuscinosis: evidence for partial response to insulin-like growth factor-1 treatment. J. Neurosci. 1999, 19:2556-2567.
10. Cooper J.D., Russell C., Mitchison H.M. Progress towards understanding disease mechanisms in small vertebrate models of neuronal ceroid lipofuscinosis. Biochim. Biophys. Acta 2006, 1762:873-889.
11. During M.J., Spencer D.D. Extracellular hippocampal glutamate and spontaneous seizure in the conscious human brain. Lancet 1993, 341:1607-1610.
12. Ankarcrona M., Dypbukt J.M., Bonfoco E., et al. Glutamate-induced neuronal death: a succession of necrosis or apoptosis depending on mitochondrial function. Neuron 1995, 15:961-973.
13. 2+ overload indicates early neuronal injury which precedes staining with viability indicators. Brain Res. 1993, 607:319-323.
14. Nicholls D.G., Budd S.L. Mitochondria and neuronal survival. Physiol. Rev. 2000, 80:315-360.
15. Jolly R.D., Brown S., Das A.M., Walkley S.U. Mitochondrial dysfunction in the neuronal ceroid-lipofuscinoses (Batten disease). Neurochem. Int. 2002, 40:565-571.
16. i triggers delayed exocytosis of ATP in cultured astrocytes. Glia 2008, 56:38-49.
17. Lacaille J.C., Mueller A.L., Kunkel D.D., Schwartzkroin P.A. Local circuit interactions between oriens/alveus interneurons and CA1 pyramidal cells in hippocampal slices: electrophysiology and morphology. J. Neurosci. 1987, 7:1979-1993.
18. Mynlieff M. Identification of different putative neuronal subtypes in cultures of the superior region of the hippocampus using electrophysiological parameters. Neuroscience 1999, 93:479-486.
19. Gandhi S., Wood-Kaczmar A., Yao Z., Plun-Favreau H., Deas E., Klupsch K., Downward J., Latchman D.S., Tabrizi S.J., Wood N.W., Duchen M.R., Abramov A.Y. PINK1-associated Parkinson's disease is caused by neuronal vulnerability to calcium-induced cell death. Mol. Cell 2009, 33:627-638.
20. Pangrsic T., Potokar M., Stenovec M., et al. Exocytotic release of ATP from cultured astrocytes. J. Biol. Chem. 2007, 282:28749-28758.
21. Verkhratsky A. Physiology and pathophysiology of the calcium store in the endoplasmic reticulum of neurons. Physiol. Rev. 2005, 85:201-279.
22. Blaustein M.P., Lederer W.J. Sodium/calcium exchange: its physiological implications. Physiol. Rev. 1999, 79:763-854.
23. 2+-ATPase inhibitor, thapsigargin,. Agents Actions 1990, 29:8-15.
24. Schatzmann H.J, Bürgin H. Calcium in human red blood cells,. Ann. N.Y. Acad. Sci. 1978, 307:125-147.
25. Khodorov B. Glutamate-induced deregulation of calcium homeostasis and mitochondrial dysfunction in mammalian central neurones. Prog. Biophys. Mol. Biol. 2004, 86:279-351.
26. Lonka L., Aalto A., Kopra O., et al. The neuronal ceroid lipofuscinosis Cln8 gene expression is developmentally regulated in mouse brain and up-regulated in the hippocampal kindling model of epilepsy. BMC Neurosci. 2005, 6:27.
27. Tyynelä J., Cooper J.D., Khan M.N., Shemilts S.J., Haltia M. Hippocampal pathology in the human neuronal ceroid-lipofuscinoses: distinct patterns of storage deposition, neurodegeneration and glial activation. Brain Pathol. 2004, 14:349-357.
28. Limbrick D.D., Churn S.B, Sombati S., DeLorenzo R.J. Inability to restore resting intracellular calcium levels as an early indicator of delayed neuronal cell death. Brain Res. 1995, 690:145-156.
29. Vergun O., Keelan J., Khodorov B.I., Duchen M.R. Glutamate-induced mitochondrial depolarisation and perturbation of calcium homeostasis in cultured rat hippocampal neurones. J. Physiol. 1999, 519:451-466.
30. Nieminen A.L., Petrie T.G., Lemasters J.J., Selman W.R. Cyclosporin A delays mitochondrial depolarization induced by N-methyl-d-aspartate in cortical neurons: evidence of the mitochondrial permeability transition. Neuroscience 1996, 75:993-997.
31. Castilho R.F., Hansson O., Ward M.W., Budd S.L., Nicholls D.G. Mitochondrial control of acute glutamate excitotoxicity in cultured cerebellar granule cells. J. Neurosci. 1998, 18:10277-10286.
32. i increase and mitochondrial depolarization induced by glutamate in cerebellar granule cells. Biochemistry (Mosc) 2006, 71:864-870.
33. 2+ liaison. Sci. STKE 2004, 215:re1.
34. Hayashi T., Rizzuto R., Hajnoczky G., Su T.P. MAM: more than just a housekeeper. Trends Cell Biol. 2009, 19:81-88.
35. Vance J.E. Phospholipid synthesis in a membrane fraction associated with mitochondria. J. Biol. Chem. 1990, 265:7248-7256.
36. Vance J.E., Stone S.J., Faust J.R. Abnormalities in mitochondria-associated membranes and phospholipid biosynthetic enzymes in the mnd/mnd mouse model of neuronal ceroid lipofuscinosis. Biochim. Biophys. Acta 1997, 1344:286-299.
37. 2+ signals into mitochondria. J. Biol. Chem. 2005, 280:40892-40900.
38. Bertamini M., Marzani B., Guarneri R., et al. Mitochondrial oxidative metabolism in motor neuron degeneration (mnd) mouse central nervous system. Eur. J. Neurosci. 2002, 16:2291-2296.
39. Hannun Y.A., Obeid L.M. Principles of bioactive lipid signalling: lessons from sphingolipids. Nat. Rev. Mol. Cell Biol. 2008, 9:139-150.
40. Winter E., Ponting C.P. TRAM LAG1 and CLN8: members of a novel family of lipid-sensing domains?. Trends Biochem. Sci. 2002, 27:381-383.
41. Riebeling C., Allegood J.C., Wang E., Merrill A.H., Futerman A.H. Two mammalian longevity assurance gene (LAG1) family members, trh1 and trh4, regulate dihydroceramide synthesis using different fatty acyl-CoA donors. J. Biol. Chem. 2003, 278:43452-43459.