메뉴 건너뛰기




Volumn 28, Issue 1, 1997, Pages 60-62

The ovine model of neuronal ceroid lipofuscinosis (NCL): Its contribution to understanding the pathogenesis of Batten disease

Author keywords

ATP synthase; Boundary lipids; Fo complex domain; Subunit c

Indexed keywords

LIPID; LYSOSOME ENZYME; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE;

EID: 0030795817     PISSN: 0174304X     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-2007-973670     Document Type: Short Survey
Times cited : (21)

References (30)
  • 3
    • 0028857502 scopus 로고
    • Specific delay of degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid-lipofuscinosis
    • Esaki, J., L. S. Wolfe, T. Higuti, K. Ishidoh, E. Kominami: Specific delay of degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid-lipofuscinosis. Neurochemistry 64 (1995) 733-741
    • (1995) Neurochemistry , vol.64 , pp. 733-741
    • Esaki, J.1    Wolfe, L.S.2    Higuti, T.3    Ishidoh, K.4    Kominami, E.5
  • 4
    • 0027312571 scopus 로고
    • Molecular basis of lysosomal accumulation of subunit c of mitochondrial ATP synthase in neuronal ceroid-lipofuscinosis
    • Ezaki, J., L. S. Wolfe, E. Kominami: Molecular basis of lysosomal accumulation of subunit c of mitochondrial ATP synthase in neuronal ceroid-lipofuscinosis J. Inherit. Metab. Dis. 16 (1993) 296-298
    • (1993) J. Inherit. Metab. Dis. , vol.16 , pp. 296-298
    • Ezaki, J.1    Wolfe, L.S.2    Kominami, E.3
  • 5
    • 0025290956 scopus 로고
    • The sequence of the major protein stored in ovine ceroid lipofuscinosis is identical with that of the dicycylohexylcarbodiimide-reactive proteolipid of mitochondrial ATP synthase
    • Fearnley, I M, J. E. Walker, R. D. Martinus, R. D. Jolly, K. B. Kirkland, G. J. Shaw, D. N. Palmer: The sequence of the major protein stored in ovine ceroid lipofuscinosis is identical with that of the dicycylohexylcarbodiimide-reactive proteolipid of mitochondrial ATP synthase. Biochem J. 268 (1990) 751-758
    • (1990) Biochem J. , vol.268 , pp. 751-758
    • Fearnley, I.M.1    Walker, J.E.2    Martinus, R.D.3    Jolly, R.D.4    Kirkland, K.B.5    Shaw, G.J.6    Palmer, D.N.7
  • 6
    • 76949131802 scopus 로고
    • Proteolipides: A new type of tissue lipoproteins
    • Folch, J., M. Lees: Proteolipides: A new type of tissue lipoproteins. J. Biol. Chem. 191 (1951) 807-817
    • (1951) J. Biol. Chem. , vol.191 , pp. 807-817
    • Folch, J.1    Lees, M.2
  • 7
    • 0022296047 scopus 로고
    • Regulation of mitochondrial protein concentration: A plausible model which may permit assessing protein turnover
    • Grisolia, S., J. Hermandez-Yago, E. Knecht: Regulation of mitochondrial protein concentration: A plausible model which may permit assessing protein turnover. Curr. Top. Cell. Regul. 27 (1985) 387-396
    • (1985) Curr. Top. Cell. Regul. , vol.27 , pp. 387-396
    • Grisolia, S.1    Hermandez-Yago, J.2    Knecht, E.3
  • 8
    • 0024313507 scopus 로고
    • Analysis of dolichyl pyrophosphoryl oligosaccharides in purified storage cytosomes from ovine ceroid-lipofuscinosis
    • Hall, N. A., R. D. Jolly, D. N. Palmer, B. D. Lake, A. D. Patrick Analysis of dolichyl pyrophosphoryl oligosaccharides in purified storage cytosomes from ovine ceroid-lipofuscinosis. Biochem. Biophys. Acta 993 (1989) 245-251
    • (1989) Biochem. Biophys. Acta , vol.993 , pp. 245-251
    • Hall, N.A.1    Jolly, R.D.2    Palmer, D.N.3    Lake, B.D.4    Patrick, A.D.5
  • 10
    • 0025101716 scopus 로고
    • Mechanisms of membrane turnover
    • Hare, J. F.: Mechanisms of membrane turnover Biochem. Biophys. Acta 1031 (1990) 71-90
    • (1990) Biochem. Biophys. Acta , vol.1031 , pp. 71-90
    • Hare, J.F.1
  • 11
    • 0024225921 scopus 로고
    • Ovine ceroid-lipofuscinosis II: Pathologic changes interpreted in light of biochemical observations
    • Jolly, R. D., A. Shimada, A. S. Craig, K. B. Kirkland, D N. Palmer: Ovine ceroid-lipofuscinosis II: Pathologic changes interpreted in light of biochemical observations Am. J. Med. Genet. Suppl. 5 (1988) 159-170
    • (1988) Am. J. Med. Genet. Suppl. , vol.5 , pp. 159-170
    • Jolly, R.D.1    Shimada, A.2    Craig, A.S.3    Kirkland, K.B.4    Palmer, D.N.5
  • 13
    • 0028932292 scopus 로고
    • Review: The neuronal ceroid-lipofuscinosis (Batten disease): Comparative Aspects
    • Jolly, R. D., D. N. Palmer: Review: The neuronal ceroid-lipofuscinosis (Batten disease): Comparative Aspects. Neuropathol Appl. Neurobiol. 21 (1995) 50-60
    • (1995) Neuropathol Appl. Neurobiol. , vol.21 , pp. 50-60
    • Jolly, R.D.1    Palmer, D.N.2
  • 14
    • 0029074322 scopus 로고
    • Canine hereditary ceroid-lipofuscinosis: Evidence for a defect in the carnitine biosynthetic pathway
    • Katz, M. L., A. N. Siakotas: Canine hereditary ceroid-lipofuscinosis: Evidence for a defect in the carnitine biosynthetic pathway Am J. Med. Genet. 57 (1995) 266-271
    • (1995) Am J. Med. Genet. , vol.57 , pp. 266-271
    • Katz, M.L.1    Siakotas, A.N.2
  • 16
    • 0029055657 scopus 로고
    • Morphological alterations in neocortical and cerebellar GABAergic neurons in a canine model of juvenile Batten disease
    • March, P. A, S. Wurzelmann, S. U. Walkley: Morphological alterations in neocortical and cerebellar GABAergic neurons in a canine model of juvenile Batten disease. Am. J. Med. Genet. 57 (1995) 204-212
    • (1995) Am. J. Med. Genet. , vol.57 , pp. 204-212
    • March, P.A.1    Wurzelmann, S.2    Walkley, S.U.3
  • 17
    • 0017816972 scopus 로고
    • Protein-immobilised lipid in dimyristoylphosphatidylcholine-substituted cytochrome oxidise: Evidence for boundary and trapped-bilayer lipid
    • Marsh, D., A. Watts, W Maschke, P F. Knowles: Protein-immobilised lipid in dimyristoylphosphatidylcholine-substituted cytochrome oxidise: Evidence for boundary and trapped-bilayer lipid. Biochem. Biophys Res. Commun. 81 (1978) 397-402
    • (1978) Biochem. Biophys Res. Commun. , vol.81 , pp. 397-402
    • Marsh, D.1    Watts, A.2    Maschke, W.3    Knowles, P.F.4
  • 18
    • 0027186335 scopus 로고
    • Characterisation of the expressed genes for subunit c of mitochondrial ATP synthase in sheep with ceroid-lipofuscinosis
    • Medd, S. M., J E. Walker, R D. Jolly: Characterisation of the expressed genes for subunit c of mitochondrial ATP synthase in sheep with ceroid-lipofuscinosis. Biochem. J. 293 (1993) 65-73
    • (1993) Biochem. J. , vol.293 , pp. 65-73
    • Medd, S.M.1    Walker, J.E.2    Jolly, R.D.3
  • 20
    • 0023038776 scopus 로고
    • Ceroid-lipofuscinosis in sheep II. The major component of the lipopigment in liver, kidney, pancreas and brain is low molecular weight protein
    • Palmer, D. N., D R. Husbands, G. Barnes, R. D. Jolly: Ceroid-lipofuscinosis in sheep II. The major component of the lipopigment in liver, kidney, pancreas and brain is low molecular weight protein. J. Biol. Chem. 261 (1986) 1773-1777
    • (1986) J. Biol. Chem. , vol.261 , pp. 1773-1777
    • Palmer, D.N.1    Husbands, D.R.2    Barnes, G.3    Jolly, R.D.4
  • 22
    • 0011333340 scopus 로고
    • Lysosomal storage of the DCCD reactive proteolipid subunit of mitochondrial ATP synthase in human and ovine ceroid lipofuscinosis
    • E. A. Porta (Ed.): New York, Plenum Press
    • Palmer, D. N., I. M. Fearnley, S. M. Medd, J. E. Walker, R. D Martinus, S. L. Bayliss et al Lysosomal storage of the DCCD reactive proteolipid subunit of mitochondrial ATP synthase in human and ovine ceroid lipofuscinosis. In: E. A. Porta (Ed.): Lipofuscin and Ceroid Pigments. New York, Plenum Press (1990) 221-233
    • (1990) Lipofuscin and Ceroid Pigments , pp. 221-233
    • Palmer, D.N.1    Fearnley, I.M.2    Medd, S.M.3    Walker, J.E.4    Martinus, R.D.5    Bayliss, S.L.6
  • 23
    • 0026539541 scopus 로고
    • Mitochondrial ATP synthase subunit c storage in the ceroid-lipofuscinosis (Batten disease)
    • 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-lipofuscinosis (Batten disease). Am. J. Med Genet. 42 (1992) 561-567
    • (1992) Am. J. Med Genet. , vol.42 , pp. 561-567
    • Palmer, D.N.1    Fearnley, I.M.2    Walker, J.E.3    Hall, N.A.4    Lake, B.D.5    Wolfe, L.S.6
  • 24
    • 8544224041 scopus 로고    scopus 로고
    • Accumulation of sphingolipid activator proteins (SAPS) A and D in granular osmiophilic deposits (GRODs) in miniature Schnauzer dogs with ceroid-lipofuscinosis
    • in press
    • Palmer, D. N., J. Tyynelä, H. C van Mil, V. J. Westlake, R. D. Jolly: Accumulation of sphingolipid activator proteins (SAPS) A and D in granular osmiophilic deposits (GRODs) in miniature Schnauzer dogs with ceroid-lipofuscinosis. J. Inherit. Metab. Dis. 19 (1996) in press
    • (1996) J. Inherit. Metab. Dis. , vol.19
    • Palmer, D.N.1    Tyynelä, J.2    Van Mil, H.C.3    Westlake, V.J.4    Jolly, R.D.5
  • 25
    • 0030845481 scopus 로고    scopus 로고
    • Different patterns of hydrophobic protein storage in different forms of neuronal ceroid lipofuscinosis (NCL, Batten disease)
    • Palmer, D. N., R. D. Jolly, H van Mil, J. Tyynela, V. J. Westlake: Different patterns of hydrophobic protein storage in different forms of neuronal ceroid lipofuscinosis (NCL, Batten disease). Neuropediatncs 28 (1997) 45-48
    • (1997) Neuropediatncs , vol.28 , pp. 45-48
    • Palmer, D.N.1    Jolly, R.D.2    Van Mil, H.3    Tyynela, J.4    Westlake, V.J.5
  • 26
    • 0029889549 scopus 로고
    • Electrospray spectrometry (ESI/MS) of ceroid lipofuscin protein: A model system for the study of Fo inhibitor interactions with mitochondrial subunit c
    • Ryan, E. M., A. Buzy, D. E. Griffiths, K R. Jennings, D. N Palmer: Electrospray spectrometry (ESI/MS) of ceroid lipofuscin protein: A model system for the study of Fo inhibitor interactions with mitochondrial subunit c Biochem. Soc. Trans. 24 (1966) 289S
    • (1966) Biochem. Soc. Trans. , vol.24
    • Ryan, E.M.1    Buzy, A.2    Griffiths, D.E.3    Jennings, K.R.4    Palmer, D.N.5
  • 27
    • 0027224115 scopus 로고
    • Storage of Saposins A and D in infantile neuronal ceroid-lipofuscinosis
    • Tyynelä, J., D. N. Palmer, M. Baumann, M Haltia: Storage of Saposins A and D in infantile neuronal ceroid-lipofuscinosis. FEBS Lett 330 (1993) 8-12
    • (1993) FEBS Lett , vol.330 , pp. 8-12
    • Tyynelä, J.1    Palmer, D.N.2    Baumann, M.3    Haltia, M.4
  • 28
    • 0029153109 scopus 로고
    • Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid-lipofuscinosis
    • Vesa, J., E. Hellsten, 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
    • (1995) Nature , vol.376 , pp. 584-587
    • Vesa, J.1    Hellsten, E.2    Verkruyse, A.3    Camp, L.A.4    Rapola, J.5    Santavuori, P.6
  • 29
    • 0027325486 scopus 로고
    • Biology of neuronal dysfunction in storage disorders
    • Walkley, S. U., P. A. March. Biology of neuronal dysfunction in storage disorders. J. Inherit. Metab. Dis. 16 (1993) 284-287
    • (1993) J. Inherit. Metab. Dis. , vol.16 , pp. 284-287
    • Walkley, S.U.1    March, P.A.2


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.