Upregulation of Bcl-2 and elevation of ceramide in Batten disease

Neuropediatrics

Volumn 28, Issue 1, 1997, Pages 37-41

  Puranam, K ^a   Qian, W H ^a   Nikbakht, K ^a   Venable, M ^a   Obeid, L ^a   Hannun, Y ^a   Boustany, R M ^a  

^a DUKE UNIVERSITY MEDICAL CENTER   (United States)

Author keywords

Apoptosis; Batten disease; Bcl 2; Ceramide; Neurodegenerative disease; Retinitis pigmentosa

Indexed keywords

CERAMIDE; RNA;

APOPTOSIS; ARTICLE; BLINDNESS; CASE REPORT; COGNITIVE DEFECT; DEGENERATIVE DISEASE; GENE EXPRESSION; HUMAN; HUMAN TISSUE; IMMUNOCYTOCHEMISTRY; MOLECULAR CLONING; MOTOR DYSFUNCTION; NERVE CELL; NEURONAL CEROID LIPOFUSCINOSIS; NORTHERN BLOTTING; PHOTORECEPTOR; PRIORITY JOURNAL; PROTO ONCOGENE; RETINITIS PIGMENTOSA; SEIZURE;

EID: 0030833658     PISSN: 0174304X     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-2007-973664     Document Type: Article

References (17)

1. Allsopp, T. E., S Wyatt, H. F. Patterson, A. M. Davies. The protooncogene Bcl-2 can selectively rescue neurotrophic-dependent neurons from apoptosis. Cell 73 (1993) 295-307
2. Boustany, R.-M., J. Alroy, E. H. Kolodny: Clinical classification of neuronal ceroid lipofuscinosis subtypes. Am. J. Med Genet. (Suppl.) 5 (1988) 47-58
3. Boustany, R.-M.: Neurodystrophies and Neurolipidoses. In: Handbook of Clinical Neurology (Eds ) Vinken P J., G. W. Bruyn, Amsterdam, Elsevier. Batten Disease or Neuronal Ceroid Lipofuscinosis. Chapter 9 (1996), in press
4. Hannun, Y. A.: The Sphingomyelin cycle and the second messenger function of ceramide. J. Biol. Chem. 269 (5) (1994) 3125-3128
5. Jarvis, W. D., R. N. Kolesnick, F. A. Fornari, R. S. Traylor, D. A. Gewritz, S. Grant: Induction of apoptotic DNA damage and cell death by activation of the sphingomyelin pathway. Proc. Natl. Acad. Sci (USA) 91 (1994) 73-77
6. Johnson, D. W, S Speier, W.-H. Qian, S. Lane, A. Cook, K. Suzuki et al: Role of subunit-9 of mitochondrial ATP synthase in Batten disease. Am. J. Med. Genet. 57 (1995) 350-360
7. Kane, D. J., T. A. Sarafian, R. Anton, H. Hahn, E. B. Gralla, J. S. Valentine et al Bcl-2 inhibition of neural death: decreased generation of reactive oxygen species. Science 262 (1993) 1274-1277
8. Lane, S. C., R. D Jolly, D. E. Schmechel, J Alroy, R.-M. Boustany: Apoptosis is the mechanism of neurodegeneration in Batten disease J. Neurochem. 67 (1996) 677-683
9. Lerner, T. J., R.-M. Boustany, J. W. Anderson, K. L. D'Argio, K. Schlumpf, A. J. Buckler et al: The International Batten Disease Consortium. Isolation of a novel gene underlying Batten disease, CLN3. Cell 82 (1995) 949-957
10. Palmer, D. N., I. M Fearnley, J. E. Walker, N. A. Hall, B. D. Lake, L. S. Wolfe et al: Mitochondrial ATP synthase subunit c storage in the ceroid lipofuscinoses (Batten disease). Am. J. Med. Genet. 42 (1992) 561-567
11. Martinou, J. C, M. Dubois-Dauphin, J. K Staple, I. Rodriguez, H. Frankowski, M. Misotten et al: Overexpression of Bcl-2 in transgenic mice protects neurons from naturally occurring cell death and experimental ischemia. Neuron. 13 (1994) 1017-1030
12. Obeid, L. M., C. M. Linardic, L. A. Karolak, Y. A. Hannun: Programmed cell death induced by ceramide. Science 259 (1993) 1769-1771
13. Oltvai, Z. N., S. J. Korsmeyer (Comment) Checkpoints of dueling dimers foil death wishes Cell 79 (1994) 109-192
14. Savukoski, M., M. Kestila, R. Williams, I. Järvelä, J Sharp, J. Harris et al: Defined chromosomal assignment of CLN5 demonstrates that at least four genetic loci are involved in the pathogenesis of human ceroid lipofuscinosis Am J. Hum Genet. 55 (1994) 695-701
15. Sebald, W., J. Hoppe: On the structure and genetics of the proteolipid subunit of the ATP synthase complex. In: Current topics in bioenergetics. Vol. 12 (1981) 1-64
16. Vesa, J., E. Hellsten, L. A. Verkruyse, L. A. Camp, J. Rapola, P. Santavuori et al: Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis. Nature 376 (1995) 584-587
17. Zhang, J., N. Alter, J. C. Reed, C. Borner, L. M. Obeid, Y. A. Hannun: Bcl-2 interrupts the ceramide mediated pathway of cell death. Proc. Natl Acad. Sci. 93 (1996) 5325-5330