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Volumn 85, Issue 15, 2007, Pages 3494-3504

Metabolomic investigation of CLN6 neuronal ceroid lipofuscinosis in affected South Hampshire sheep

Author keywords

Batten disease; Brain; CLN6; CSF; Glutamate glutamine cycling; Metabonomics; Multivariate statistical analysis; Neurodegenerative disease

Indexed keywords

4 AMINOBUTYRIC ACID; ACETIC ACID; ASPARTIC ACID; GLUTAMIC ACID; GLUTAMINE; INOSITOL; N ACETYLASPARTIC ACID; SUCCINIC ACID;

EID: 35848957261     PISSN: 03604012     EISSN: 10974547     Source Type: Journal    
DOI: 10.1002/jnr.21343     Document Type: Conference Paper
Times cited : (21)

References (37)
  • 1
    • 0036345741 scopus 로고    scopus 로고
    • Impaired glutamate transport and glutamate-glutamine cycling: Downstream effects of the Huntington mutation
    • Behrens PF, Franz P, Woodman B, Lindenberg KS, Landwehrmeyer GB. 2002. Impaired glutamate transport and glutamate-glutamine cycling: downstream effects of the Huntington mutation. Brain 125:1908-1922.
    • (2002) Brain , vol.125 , pp. 1908-1922
    • Behrens, P.F.1    Franz, P.2    Woodman, B.3    Lindenberg, K.S.4    Landwehrmeyer, G.B.5
  • 2
    • 0027749707 scopus 로고
    • Multinuclear NMR studies on the energy metabolism of glial and neuronal cells
    • Brand A, Richter-Landsberg C, Leibfritz D. 1993. Multinuclear NMR studies on the energy metabolism of glial and neuronal cells. Dev Neurosci 15:289-298.
    • (1993) Dev Neurosci , vol.15 , pp. 289-298
    • Brand, A.1    Richter-Landsberg, C.2    Leibfritz, D.3
  • 3
    • 0031846129 scopus 로고    scopus 로고
    • Ovine neuronal ceroid lipofuscinosis: A large animal model syntenic with the human neuronal ceroid lipofuscinosis variant CLN6
    • Broom MF, Zhou C, Broom JE, Barwell KJ, Jolly RD, Hill DF. 1998. Ovine neuronal ceroid lipofuscinosis: a large animal model syntenic with the human neuronal ceroid lipofuscinosis variant CLN6. J Med Genet 35:717-721.
    • (1998) J Med Genet , vol.35 , pp. 717-721
    • Broom, M.F.1    Zhou, C.2    Broom, J.E.3    Barwell, K.J.4    Jolly, R.D.5    Hill, D.F.6
  • 6
    • 28044450369 scopus 로고    scopus 로고
    • Proton nuclear magnetic resonance-based metabonomics for rapid diagnosis of meningitis and ventriculitis
    • Coen M, O'Sullivan M, Bubb WA, Kuchel PW, Sorrell T. 2005. Proton nuclear magnetic resonance-based metabonomics for rapid diagnosis of meningitis and ventriculitis. Clin Infect Dis 41:1582-1590.
    • (2005) Clin Infect Dis , vol.41 , pp. 1582-1590
    • Coen, M.1    O'Sullivan, M.2    Bubb, W.A.3    Kuchel, P.W.4    Sorrell, T.5
  • 7
    • 0037399422 scopus 로고    scopus 로고
    • Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosis
    • Cooper JD. 2003. Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosis. Curr Opin Neurol 16:121-128.
    • (2003) Curr Opin Neurol , vol.16 , pp. 121-128
    • Cooper, J.D.1
  • 9
    • 0025290956 scopus 로고
    • The sequence of the major protein stored in ovine ceroid lipofuscinosis is identical with that of the dicyclohexylcarbodiimide-reactive proteolipid of mitochondrial ATP synthase
    • Fearnley IM, Walker JE, Martinus RD, Jolly RD, Kirkland KB, Shaw GJ, Palmer DN. 1990. The sequence of the major protein stored in ovine ceroid lipofuscinosis is identical with that of the dicyclohexylcarbodiimide-reactive proteolipid of mitochondrial ATP synthase. Biochem J 268:751-758.
    • (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
  • 11
    • 0034099574 scopus 로고    scopus 로고
    • Proton NMR chemical shifts and coupling constants for brain metabolites
    • Govindaraju V, Young K, Maudsley AA. 2000. Proton NMR chemical shifts and coupling constants for brain metabolites. NMR Biomed 13:129-153.
    • (2000) NMR Biomed , vol.13 , pp. 129-153
    • Govindaraju, V.1    Young, K.2    Maudsley, A.A.3
  • 12
    • 3242741850 scopus 로고    scopus 로고
    • Design of experiments: An efficient strategy to identify factors influencing extraction and derivatization of Arabidopsis thaliana samples in metabolomic studies with gas chromatography/mass spectrometry
    • Gullberg J, Jonsson P, Nordstrom A, Sjostrom M, Moritz T. 2004. Design of experiments: an efficient strategy to identify factors influencing extraction and derivatization of Arabidopsis thaliana samples in metabolomic studies with gas chromatography/mass spectrometry. Anal Biochem 331:283-295.
    • (2004) Anal Biochem , vol.331 , pp. 283-295
    • Gullberg, J.1    Jonsson, P.2    Nordstrom, A.3    Sjostrom, M.4    Moritz, T.5
  • 13
    • 0037226939 scopus 로고    scopus 로고
    • The neuronal ceroid-lipofuscinoses
    • Haltia M. 2003. The neuronal ceroid-lipofuscinoses. J Neuropathol Exp Neurol 62:1-13.
    • (2003) J Neuropathol Exp Neurol , vol.62 , pp. 1-13
    • Haltia, M.1
  • 14
    • 33750986182 scopus 로고    scopus 로고
    • The neuronal ceroid-lipofuscinoses: From past to present
    • Haltia M. 2006. The neuronal ceroid-lipofuscinoses: from past to present. Biochim Biophys Acta 1762:850-856.
    • (2006) Biochim Biophys Acta , vol.1762 , pp. 850-856
    • Haltia, M.1
  • 16
    • 0024336199 scopus 로고
    • Ceroid-lipofuscinosis (Batten's disease): Pathogenesis and sequential neuropathological changes in the ovine model
    • Jolly RD, Shimada A, Dopfmer I, Slack PM, Birtles MJ, Palmer DN. 1989. Ceroid-lipofuscinosis (Batten's disease): pathogenesis and sequential neuropathological changes in the ovine model. Neuropathol Appl Neurobiol 15:371-383.
    • (1989) Neuropathol Appl Neurobiol , vol.15 , pp. 371-383
    • Jolly, R.D.1    Shimada, A.2    Dopfmer, I.3    Slack, P.M.4    Birtles, M.J.5    Palmer, D.N.6
  • 17
    • 33745018230 scopus 로고    scopus 로고
    • Activation of non-neuronal cells within the prenatal developing brain of sheep with neuronal ceroid lipofuscinosis
    • Kay GW, Palmer DN, Rezaie P, Cooper JD. 2006. Activation of non-neuronal cells within the prenatal developing brain of sheep with neuronal ceroid lipofuscinosis. Brain Pathol 16:110-116.
    • (2006) Brain Pathol , vol.16 , pp. 110-116
    • Kay, G.W.1    Palmer, D.N.2    Rezaie, P.3    Cooper, J.D.4
  • 20
    • 0027320272 scopus 로고
    • Nuclear magnetic resonance study of cerebrospinal fluid from patients with multiple sclerosis
    • Lynch J, Peeling J, Auty A, Sutherland GR. 1993. Nuclear magnetic resonance study of cerebrospinal fluid from patients with multiple sclerosis. Can J Neurol Sci 20:194-198.
    • (1993) Can J Neurol Sci , vol.20 , pp. 194-198
    • Lynch, J.1    Peeling, J.2    Auty, A.3    Sutherland, G.R.4
  • 21
    • 49049150378 scopus 로고
    • Two-dimensional chemical exchange and cross-relaxation spectroscopy of coupled nuclear spins
    • Macura S, Huang ,Y. 1981. Two-dimensional chemical exchange and cross-relaxation spectroscopy of coupled nuclear spins. J Magn Reson 43:259-281.
    • (1981) J Magn Reson , vol.43 , pp. 259-281
    • Macura, S.1    Huang, Y.2
  • 22
    • 0022367967 scopus 로고
    • Ceroid-lipofuscinosis (Batten's disease): Pathogenesis of blindness in the ovine model
    • Mayhew IG, Jolly RD, Pickett BT, Slack PM. 1985. Ceroid-lipofuscinosis (Batten's disease): pathogenesis of blindness in the ovine model. Neuropathol Appl Neurobiol 11:273-290.
    • (1985) Neuropathol Appl Neurobiol , vol.11 , pp. 273-290
    • Mayhew, I.G.1    Jolly, R.D.2    Pickett, B.T.3    Slack, P.M.4
  • 23
    • 0035684428 scopus 로고    scopus 로고
    • Neurodegenerative disease: The neuronal ceroid lipofuscinoses (Batten disease)
    • Mitchison HM, Mole SE. 2001. Neurodegenerative disease: the neuronal ceroid lipofuscinoses (Batten disease). Curr Opin Neurol 14:795-803.
    • (2001) Curr Opin Neurol , vol.14 , pp. 795-803
    • Mitchison, H.M.1    Mole, S.E.2
  • 24
    • 1042276701 scopus 로고    scopus 로고
    • Selectivity and types of cell death in the neuronal ceroid lipofuscinoses
    • Mitchison HM, Lim MJ, Cooper JD. 2004. Selectivity and types of cell death in the neuronal ceroid lipofuscinoses. Brain Pathol 14:86-96.
    • (2004) Brain Pathol , vol.14 , pp. 86-96
    • Mitchison, H.M.1    Lim, M.J.2    Cooper, J.D.3
  • 25
    • 0034923779 scopus 로고    scopus 로고
    • Changes in GABAergic neuron distribution in situ and in neuron cultures in ovine (OCL6) Batten disease
    • Oswald MJ, Kay GW, Palmer DN. 2001. Changes in GABAergic neuron distribution in situ and in neuron cultures in ovine (OCL6) Batten disease. Eur J Paediatr Neurol 5(Suppl A):135-142.
    • (2001) Eur J Paediatr Neurol , vol.5 , Issue.SUPPL. A , pp. 135-142
    • Oswald, M.J.1    Kay, G.W.2    Palmer, D.N.3
  • 26
    • 24044496235 scopus 로고    scopus 로고
    • Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6)
    • Oswald MJ, Palmer DN, Kay GW, Shemilt SJA, Rezaie P, Cooper JD. 2005. Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6). Neurobiol Dis 20:49-63.
    • (2005) Neurobiol Dis , vol.20 , pp. 49-63
    • Oswald, M.J.1    Palmer, D.N.2    Kay, G.W.3    Shemilt, S.J.A.4    Rezaie, P.5    Cooper, J.D.6
  • 27
    • 0024511783 scopus 로고
    • Ovine ceroid lipofuscinosis. The major lipopigment protein and the lipid-binding subunit of mitochondrial ATP synthase have the same NH2-terminal sequence
    • Palmer DN, Martinus RD, Cooper SM, Midwinter GG, Reid JC, Jolly RD. 1989. Ovine ceroid lipofuscinosis. The major lipopigment protein and the lipid-binding subunit of mitochondrial ATP synthase have the same NH2-terminal sequence. J Biol Chem 264:5736-5740.
    • (1989) J Biol Chem , vol.264 , pp. 5736-5740
    • Palmer, D.N.1    Martinus, R.D.2    Cooper, S.M.3    Midwinter, G.G.4    Reid, J.C.5    Jolly, R.D.6
  • 29
    • 0030845481 scopus 로고    scopus 로고
    • Different patterns of hydrophobic protein storage in different forms of neuronal ceroid lipofuscinosis (NCL, Batten disease)
    • Palmer DN, Jolly RD, van Mil HC, Tyynelä J, Westlake VJ. 1997. Different patterns of hydrophobic protein storage in different forms of neuronal ceroid lipofuscinosis (NCL, Batten disease). Neuropediatrics 28:45-48.
    • (1997) Neuropediatrics , vol.28 , pp. 45-48
    • Palmer, D.N.1    Jolly, R.D.2    van Mil, H.C.3    Tyynelä, J.4    Westlake, V.J.5
  • 30
    • 35848935487 scopus 로고    scopus 로고
    • 1H NMR analysis of cerebrospinal fluid: Applications to diseases affecting the brain
    • 1H NMR analysis of cerebrospinal fluid: applications to diseases affecting the brain. Proc Int Soc Magn Reson Med 8:1147.
    • (2000) Proc Int Soc Magn Reson Med , vol.8 , pp. 1147
    • Parkes, H.1
  • 33
    • 0024399472 scopus 로고
    • Time course of CSF lactate level in subarachnoid haemorrhage. Correlation with clinical grading and prognosis
    • Shimoda M, Yamada S, Yamamoto I, Tsugane R, Sato O. 1989. Time course of CSF lactate level in subarachnoid haemorrhage. Correlation with clinical grading and prognosis. Acta Neurochir 99:127-134.
    • (1989) Acta Neurochir , vol.99 , pp. 127-134
    • Shimoda, M.1    Yamada, S.2    Yamamoto, I.3    Tsugane, R.4    Sato, O.5
  • 34
    • 33750180119 scopus 로고    scopus 로고
    • A missense mutation (c,184C→T) in ovine CLN6 causes neuronal ceroid lipofuscinosis in Merino sheep whereas South Hampshire sheep have reduced levels of CLN6 mRNA
    • in press
    • Tammen I. 2006. A missense mutation (c,184C→T) in ovine CLN6 causes neuronal ceroid lipofuscinosis in Merino sheep whereas South Hampshire sheep have reduced levels of CLN6 mRNA. Biochim Biophys Acta (in press).
    • (2006) Biochim Biophys Acta
    • Tammen, I.1
  • 35
    • 33750180119 scopus 로고    scopus 로고
    • A missense mutation (c. 184c→T) in ovine CLN6 causes neuronal ceroid lipofuscinosis in Australian Merino sheep whereas affected New Zealand South Hampshire sheep have reduced levels of mRNA
    • Tammen I, Houweling PJ, Frugier T, Mitchell NL, Kay GW, Cavanagh JAL, Cook RW, Raadsma HW, Palmer DN. 2006. A missense mutation (c. 184c→T) in ovine CLN6 causes neuronal ceroid lipofuscinosis in Australian Merino sheep whereas affected New Zealand South Hampshire sheep have reduced levels of mRNA. Biochim et Biophysica Acta 1762:898-905.
    • (2006) Biochim et Biophysica Acta , vol.1762 , pp. 898-905
    • Tammen, I.1    Houweling, P.J.2    Frugier, T.3    Mitchell, N.L.4    Kay, G.W.5    Cavanagh, J.A.L.6    Cook, R.W.7    Raadsma, H.W.8    Palmer, D.N.9
  • 36
    • 33746615101 scopus 로고    scopus 로고
    • Changes in concentration of cerebrospinal fluid components in patients with traumatic brain injury
    • Toczylowska B, Chalimoniuk M, Wodowska M, Mayzner-Zawadzk E. 2006. Changes in concentration of cerebrospinal fluid components in patients with traumatic brain injury. Brain Res 1104:183-189.
    • (2006) Brain Res , vol.1104 , pp. 183-189
    • Toczylowska, B.1    Chalimoniuk, M.2    Wodowska, M.3    Mayzner-Zawadzk, E.4
  • 37
    • 35848968336 scopus 로고    scopus 로고
    • Saftig P, editor. Austin, TX: Landes Bioscience. p
    • Tyynelä J. 2004. Neuronal ceroid-lipofuscinoses. In: Saftig P, editor. Austin, TX: Landes Bioscience. p 82-99.
    • (2004) Neuronal ceroid-lipofuscinoses , pp. 82-99
    • Tyynelä, J.1


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.