-
2
-
-
33846303512
-
Structure of aspartoacylase, the brain enzyme impaired in Canavan disease
-
E. Bitto, C.A. Bingman, G.E. Wesenberg, J.G. McCoy, and G.N. Phillips Structure of aspartoacylase, the brain enzyme impaired in Canavan disease PNAS 104 2007 456 461
-
(2007)
PNAS
, vol.104
, pp. 456-461
-
-
Bitto, E.1
Bingman, C.A.2
Wesenberg, G.E.3
McCoy, J.G.4
Phillips, G.N.5
-
4
-
-
33846256312
-
1H-MRS) and diffusion-weighted MRI
-
1H-MRS) and diffusion-weighted MRI Neuropediatrics 37 2006 209 221
-
(2006)
Neuropediatrics
, vol.37
, pp. 209-221
-
-
Janson, C.G.1
McPhee, S.W.J.2
Francis, J.3
Shera, D.4
Assadi, M.5
Freese, A.6
Hurh, P.7
Haselgrove, J.8
Wang, D.J.9
Bilaniuk, L.10
Leone, P.11
-
5
-
-
0034915929
-
Canavan disease: A review of recent developments
-
N. Gordon Canavan disease: a review of recent developments Eur. J. Paediatr. Neurol. 5 2000 65 69
-
(2000)
Eur. J. Paediatr. Neurol.
, vol.5
, pp. 65-69
-
-
Gordon, N.1
-
6
-
-
0346787546
-
Canavan disease: Carrier-frequency determination in the ashkenazi jewish population and development of a novel molecular diagnostic assay
-
A. Feigenbaum, R. Moore, J. Clarke, S. Hewson, D. Chitayat, P.N. Ray, and T.L. Stockley Canavan disease: carrier-frequency determination in the ashkenazi jewish population and development of a novel molecular diagnostic assay Am. J. Med. Genet. 124A 2004 142 147
-
(2004)
Am. J. Med. Genet.
, vol.124 A
, pp. 142-147
-
-
Feigenbaum, A.1
Moore, R.2
Clarke, J.3
Hewson, S.4
Chitayat, D.5
Ray, P.N.6
Stockley, T.L.7
-
7
-
-
0141872257
-
Schilders encephalitis periaxialis diffusa. Report of a case in a child aged sixteen and one-half months
-
M.M. Canavan Schilders encephalitis periaxialis diffusa. Report of a case in a child aged sixteen and one-half months Arch. Neurol. Psychiat. 25 1931 299 308
-
(1931)
Arch. Neurol. Psychiat.
, vol.25
, pp. 299-308
-
-
Canavan, M.M.1
-
8
-
-
0031243417
-
Canavan disease: Analysis of the nature of the metabolic lesions responsible for development of the observed clinical symptoms
-
M.H. Baslow, and T.R. Resnik Canavan disease: analysis of the nature of the metabolic lesions responsible for development of the observed clinical symptoms J. Mol. Neurosci. 9 1997 109 126
-
(1997)
J. Mol. Neurosci.
, vol.9
, pp. 109-126
-
-
Baslow, M.H.1
Resnik, T.R.2
-
9
-
-
0032968640
-
The spectrum of mutations of the aspartoacylase gene in Canavan disease in non-jewish patients
-
O.N. Elpeleg, and A. Shaag The spectrum of mutations of the aspartoacylase gene in Canavan disease in non-jewish patients J. Inherit. Metab. Dis. 22 1999 531 534
-
(1999)
J. Inherit. Metab. Dis.
, vol.22
, pp. 531-534
-
-
Elpeleg, O.N.1
Shaag, A.2
-
11
-
-
0034485963
-
Canavan's spongiform leukodystrophy: A clinical anatomy of a genetic metabolic CNS disease. An analytical review
-
M.H. Baslow Canavan's spongiform leukodystrophy: a clinical anatomy of a genetic metabolic CNS disease. An analytical review J. Mol. Neurosci. 15 2000 61 69
-
(2000)
J. Mol. Neurosci.
, vol.15
, pp. 61-69
-
-
Baslow, M.H.1
-
12
-
-
0036881453
-
Identification and characterization of novel mutations of aspartoacylase gene in non-Jewish patients with Canavan disease
-
B.J. Zeng, Z.H. Wang, L.A. Ribiero, P. Leone, R. DeGasperi, S.J. Kim, S. Raghavan, E. Ong, G.M. Pastores, and E.H. Kolodny Identification and characterization of novel mutations of aspartoacylase gene in non-Jewish patients with Canavan disease J. Inherit. Metab. Dis. 25 2002 557 570
-
(2002)
J. Inherit. Metab. Dis.
, vol.25
, pp. 557-570
-
-
Zeng, B.J.1
Wang, Z.H.2
Ribiero, L.A.3
Leone, P.4
Degasperi, R.5
Kim, S.J.6
Raghavan, S.7
Ong, E.8
Pastores, G.M.9
Kolodny, E.H.10
-
13
-
-
77953230252
-
Lithium citrate reduces excessive intra-cerebral N-acetylaspartate in Canavan disease
-
M. Assadi, C. Janson, D.-J. Wang, O. Goldfarb, N. Suri, L. Bilaniuk, and P. Leone Lithium citrate reduces excessive intra-cerebral N-acetylaspartate in Canavan disease Eur. J. Paediatr. Neurol. 14 2010 354 359
-
(2010)
Eur. J. Paediatr. Neurol.
, vol.14
, pp. 354-359
-
-
Assadi, M.1
Janson, C.2
Wang, D.-J.3
Goldfarb, O.4
Suri, N.5
Bilaniuk, L.6
Leone, P.7
-
15
-
-
0036162622
-
Evidence supporting a role for N-acetyl-l-aspartate as a molecular water pump in myelinated neurons in the central nervous system. An analytical review
-
M.H. Baslow Evidence supporting a role for N-acetyl-l-aspartate as a molecular water pump in myelinated neurons in the central nervous system. An analytical review Neurochem. Int. 40 2002 295 300
-
(2002)
Neurochem. Int.
, vol.40
, pp. 295-300
-
-
Baslow, M.H.1
-
16
-
-
84900604298
-
N-acetylaspartate and N-acetylaspartylglutamate
-
A. Lajtha, third ed. Amino Acids and Peptides in the Nervous System Springer Sci. (Chapter 14), 305-346
-
M.H. Baslow N-acetylaspartate and N-acetylaspartylglutamate A. Lajtha, Handbook of Neurochemistry and Molecular Neurobiology third ed. Amino Acids and Peptides in the Nervous System vol 6 2007 Springer Sci. 418 (Chapter 14), 305-346
-
(2007)
Handbook of Neurochemistry and Molecular Neurobiology
, vol.6
, pp. 418
-
-
Baslow, M.H.1
-
17
-
-
76449107398
-
A novel key-lock mechanism for inactivating amino acid neurotransmitters during transit across extracellular space
-
M.H. Baslow A novel key-lock mechanism for inactivating amino acid neurotransmitters during transit across extracellular space Amino Acids 38 2010 51 55
-
(2010)
Amino Acids
, vol.38
, pp. 51-55
-
-
Baslow, M.H.1
-
18
-
-
79960734637
-
Extensive aspartoacylase expression in the rat central nervous system
-
J.R. Moffett, P. Arun, P.S. Ariyannur, J.Y. Garbern, D.M. Jacobowitz, and A.M.A. Namboodiri Extensive aspartoacylase expression in the rat central nervous system Glia 59 2011 1414 1434
-
(2011)
Glia
, vol.59
, pp. 1414-1434
-
-
Moffett, J.R.1
Arun, P.2
Ariyannur, P.S.3
Garbern, J.Y.4
Jacobowitz, D.M.5
Namboodiri, A.M.A.6
-
19
-
-
0037115078
-
Effect of N-acetylaspartic acid on the diffusion coefficient of water: A proton magnetic resonance phantom method for measurement of osmolyte-obligated water
-
M.H. Baslow, and D.N. Guilfoyle Effect of N-acetylaspartic acid on the diffusion coefficient of water: a proton magnetic resonance phantom method for measurement of osmolyte-obligated water Analyt. Biochem. 311 2002 133 138
-
(2002)
Analyt. Biochem.
, vol.311
, pp. 133-138
-
-
Baslow, M.H.1
Guilfoyle, D.N.2
-
20
-
-
34548051745
-
Behavior of acetyl modified amino acids in reverse micelles: A non-invasive and physiochemical approach
-
S.K. Mehta, K. Kaur, S. Sharma, and K.K. Bhasin Behavior of acetyl modified amino acids in reverse micelles: a non-invasive and physiochemical approach J. Colloid Interface Sci. 314 2007 689 698
-
(2007)
J. Colloid Interface Sci.
, vol.314
, pp. 689-698
-
-
Mehta, S.K.1
Kaur, K.2
Sharma, S.3
Bhasin, K.K.4
-
21
-
-
68349141082
-
Are astrocytes the missing link between lack of brain aspartoacylase activity and the spongiform leukodystrophy in Canavan disease?
-
M.H. Baslow, and D.N. Guilfoyle Are astrocytes the missing link between lack of brain aspartoacylase activity and the spongiform leukodystrophy in Canavan disease? Neurochem. Res. 34 2009 1523 1534
-
(2009)
Neurochem. Res.
, vol.34
, pp. 1523-1534
-
-
Baslow, M.H.1
Guilfoyle, D.N.2
-
22
-
-
34047147420
-
Bimodal occurrence of aspartoacylase in myelin and cytosol of brain
-
J. Wang, R. Matalon, B. Gita, W. Gusheng, L. Hong, L. Tong, L. Zi-Hua, and R.W. Ledeen Bimodal occurrence of aspartoacylase in myelin and cytosol of brain J. Neurochem. 101 2007 448 457
-
(2007)
J. Neurochem.
, vol.101
, pp. 448-457
-
-
Wang, J.1
Matalon, R.2
Gita, B.3
Gusheng, W.4
Hong, L.5
Tong, L.6
Zi-Hua, L.7
Ledeen, R.W.8
-
23
-
-
0347519132
-
Feeding versus infusion: A novel approach to study the NAA metabolism in rat brain
-
G. Karelson, A. Ziegler, B. Kunnecke, and J. Seelig Feeding versus infusion: a novel approach to study the NAA metabolism in rat brain NMR Biomed. 16 2003 413 423
-
(2003)
NMR Biomed.
, vol.16
, pp. 413-423
-
-
Karelson, G.1
Ziegler, A.2
Kunnecke, B.3
Seelig, J.4
-
24
-
-
0028292408
-
Extracellular N-acetylaspartate in rat brain: In vivo determination of basal levels and changes evoked by high K+
-
D.L. Taylor, S.E.C. Davies, T.P. Obrenovitch, J. Urenjak, D.A. Richards, J.B. Clark, and L. Symon Extracellular N-acetylaspartate in rat brain: in vivo determination of basal levels and changes evoked by high K+ J. Neurochem. 62 1994 2349 2355
-
(1994)
J. Neurochem.
, vol.62
, pp. 2349-2355
-
-
Taylor, D.L.1
Davies, S.E.C.2
Obrenovitch, T.P.3
Urenjak, J.4
Richards, D.A.5
Clark, J.B.6
Symon, L.7
-
25
-
-
0031031171
-
Transient elevation of interstitial N-acetylaspartate in reversible global brain ischemia
-
T.N. Sager, A. Fink-Jensen, and A.J. Hansen Transient elevation of interstitial N-acetylaspartate in reversible global brain ischemia J. Neurochem. 68 1997 675 682
-
(1997)
J. Neurochem.
, vol.68
, pp. 675-682
-
-
Sager, T.N.1
Fink-Jensen, A.2
Hansen, A.J.3
-
26
-
-
0015611504
-
The occurrence of N-acetylaspartate amidohydrolase (aminoacylase II) in the developing rat
-
A.F. D'Adamo, J.C. Smith, and C. Woiler The occurrence of N-acetylaspartate amidohydrolase (aminoacylase II) in the developing rat J. Neurochem. 20 1973 1275 1278
-
(1973)
J. Neurochem.
, vol.20
, pp. 1275-1278
-
-
D'Adamo, A.F.1
Smith, J.C.2
Woiler, C.3
-
27
-
-
0029034599
-
Changes in N-acetyl-aspartate content during focal and global brain ischemia of the rat
-
T.N. Sager, H. Laursen, and A.J. Hansen Changes in N-acetyl-aspartate content during focal and global brain ischemia of the rat J. Cereb. Blood Flow Metab. 15 1995 639 646
-
(1995)
J. Cereb. Blood Flow Metab.
, vol.15
, pp. 639-646
-
-
Sager, T.N.1
Laursen, H.2
Hansen, A.J.3
-
28
-
-
0344069638
-
Brain adaptation to water loading in rabbits as assessed by NMR relaxometry
-
Z. Vajda, E. Berenyi, P. Bogner, I. Repa, T. Doczi, and E. Sulyok Brain adaptation to water loading in rabbits as assessed by NMR relaxometry Pediatr. Res. 46 1999 450 459
-
(1999)
Pediatr. Res.
, vol.46
, pp. 450-459
-
-
Vajda, Z.1
Berenyi, E.2
Bogner, P.3
Repa, I.4
Doczi, T.5
Sulyok, E.6
-
29
-
-
0017293461
-
Amidohydrolases of brain; Enzymatic hydrolysis of N-acetyl-l-aspartate and other N-acyl-l-amino acids
-
F.B. Goldstein Amidohydrolases of brain; enzymatic hydrolysis of N-acetyl-l-aspartate and other N-acyl-l-amino acids J. Neurochem. 26 1976 45 49
-
(1976)
J. Neurochem.
, vol.26
, pp. 45-49
-
-
Goldstein, F.B.1
-
30
-
-
0035707750
-
Differential expression of carnosine, homocarnosine and N-acetyl-l-histidine hydrolytic activities in cultured rat macroglial cells
-
M.H. Baslow, R.F. Suckow, M.J. Berg, N. Marks, M. Saito, and K.K. Bhakoo Differential expression of carnosine, homocarnosine and N-acetyl-l-histidine hydrolytic activities in cultured rat macroglial cells J. Mol. Neurosci. 17 2001 351 359
-
(2001)
J. Mol. Neurosci.
, vol.17
, pp. 351-359
-
-
Baslow, M.H.1
Suckow, R.F.2
Berg, M.J.3
Marks, N.4
Saito, M.5
Bhakoo, K.K.6
-
31
-
-
0025359148
-
Analysis of carnosine, homocarnosine, and other histidyl derivatives in rat brain
-
J.J. O'Dowd, M.T. Cairns, M. Trainor, D.J. Robins, and D.J. Miller Analysis of carnosine, homocarnosine, and other histidyl derivatives in rat brain J. Neurochem. 55 1990 446 452
-
(1990)
J. Neurochem.
, vol.55
, pp. 446-452
-
-
O'Dowd, J.J.1
Cairns, M.T.2
Trainor, M.3
Robins, D.J.4
Miller, D.J.5
-
32
-
-
0025092193
-
Histidyl derivatives in rabbit lens and their diminution in human cataract
-
J.L. Jay, D.J. Miller, J.D. Morrison, and J.J. O'Dowd Histidyl derivatives in rabbit lens and their diminution in human cataract J. Physiol. 420 1990 155
-
(1990)
J. Physiol.
, vol.420
, pp. 155
-
-
Jay, J.L.1
Miller, D.J.2
Morrison, J.D.3
O'Dowd, J.J.4
-
33
-
-
0023757196
-
Detection, characterisation, and quantification of carnosine and other histidyl derivatives in cardiac and skeletal muscle
-
J.J. O'Dowd, D.J. Robins, and D.J. Miller Detection, characterisation, and quantification of carnosine and other histidyl derivatives in cardiac and skeletal muscle Biochim. Biophys. Acta 967 1988 241 249
-
(1988)
Biochim. Biophys. Acta
, vol.967
, pp. 241-249
-
-
O'Dowd, J.J.1
Robins, D.J.2
Miller, D.J.3
-
34
-
-
18144381244
-
Transport characteristics of N-acetyl-l-aspartate in rat astrocytes: Involvement of sodium-coupled high-affinity carboxylate transporter NaC3/NaDC3-mediated transport system
-
T. Fujita, H. Katsukawa, E. Yodoya, M. Wada, A. Shimada, N. Okada, A. Yamamoto, and V. Ganapathy Transport characteristics of N-acetyl-l-aspartate in rat astrocytes: involvement of sodium-coupled high-affinity carboxylate transporter NaC3/NaDC3-mediated transport system J. Neurochem. 93 2005 706 714
-
(2005)
J. Neurochem.
, vol.93
, pp. 706-714
-
-
Fujita, T.1
Katsukawa, H.2
Yodoya, E.3
Wada, M.4
Shimada, A.5
Okada, N.6
Yamamoto, A.7
Ganapathy, V.8
-
35
-
-
33646165531
-
Decreased renal organic anion secretion and plasma accumulation of endogenous organic anions in OAT1 knock-out mice
-
S.A. Eraly, V. Vallon, D.A. Vaughn, J.A. Gangoiti, K. Richter, M. Nagle, J.C. Monte, T. Rieg, D.M. Truong, J.M. Long, B.A. Barshop, G. Kaler, and S.K. Nigam Decreased renal organic anion secretion and plasma accumulation of endogenous organic anions in OAT1 knock-out mice J. Biol. Chem. 281 2006 5072 5083
-
(2006)
J. Biol. Chem.
, vol.281
, pp. 5072-5083
-
-
Eraly, S.A.1
Vallon, V.2
Vaughn, D.A.3
Gangoiti, J.A.4
Richter, K.5
Nagle, M.6
Monte, J.C.7
Rieg, T.8
Truong, D.M.9
Long, J.M.10
Barshop, B.A.11
Kaler, G.12
Nigam, S.K.13
-
36
-
-
79960729662
-
Endogenous aspartoacylase expression is responsive to glutamatergic activity in vitro and in vivo
-
J.S. Francis, L. Strande, A. Pu, and P. Leone Endogenous aspartoacylase expression is responsive to glutamatergic activity in vitro and in vivo Glia 59 2011 1435 1446
-
(2011)
Glia
, vol.59
, pp. 1435-1446
-
-
Francis, J.S.1
Strande, L.2
Pu, A.3
Leone, P.4
-
37
-
-
84874658328
-
N-acetylaspartate metabolism underlays the structural and functional units of the vertebrate brain. A bioenergetic rationale for clinical observations of changes in the neuronal biomarker "nAA" in many human brain disorders
-
10.4172/beg.1000102
-
M.H. Baslow, and A.P. Burlina N-acetylaspartate metabolism underlays the structural and functional units of the vertebrate brain. A bioenergetic rationale for clinical observations of changes in the neuronal biomarker "NAA" in many human brain disorders Bioenergetics Open Access 1 2012 10.4172/beg.1000102
-
(2012)
Bioenergetics Open Access
, vol.1
-
-
Baslow, M.H.1
Burlina, A.P.2
-
38
-
-
0032899289
-
Molecular water pumps and the etiology of Canavan disease; A case of the sorcerer's apprentice
-
M.H. Baslow Molecular water pumps and the etiology of Canavan disease; a case of the sorcerer's apprentice J. Inherit. Metab. Dis. 22 1999 99 101
-
(1999)
J. Inherit. Metab. Dis.
, vol.22
, pp. 99-101
-
-
Baslow, M.H.1
-
39
-
-
0032055626
-
The human Na+-glucose cotransporter is a molecular water pump
-
A.-K. Meinild, D.A. Klaerke, D.D.F. Loo, E.M. Wright, and T. Zeuthen The human Na+-glucose cotransporter is a molecular water pump J. Physiol. 508 1998 15 21
-
(1998)
J. Physiol.
, vol.508
, pp. 15-21
-
-
Meinild, A.-K.1
Klaerke, D.A.2
Loo, D.D.F.3
Wright, E.M.4
Zeuthen, T.5
-
40
-
-
0031722757
-
Function of the N-acetyl-l-histidine system in the vertebrate eye. Evidence in support of a role as a molecular water pump
-
M.H. Baslow Function of the N-acetyl-l-histidine system in the vertebrate eye. Evidence in support of a role as a molecular water pump J. Mol. Neurosci. 10 1998 193 208
-
(1998)
J. Mol. Neurosci.
, vol.10
, pp. 193-208
-
-
Baslow, M.H.1
-
41
-
-
0036661083
-
Aquaporin water channels-from atomic structure to clinical medicine
-
P. Agre, L.S. King, M. Yasui, W.B. Guggino, O.P. Ottersen, Y. Fujiyoshi, A. Engel, and S. Neilsen Aquaporin water channels-from atomic structure to clinical medicine J. Physiol. 542 2002 3 16
-
(2002)
J. Physiol.
, vol.542
, pp. 3-16
-
-
Agre, P.1
King, L.S.2
Yasui, M.3
Guggino, W.B.4
Ottersen, O.P.5
Fujiyoshi, Y.6
Engel, A.7
Neilsen, S.8
-
42
-
-
77953664158
-
Aquaporin biology and nervous system
-
B. Buffoli Aquaporin biology and nervous system Curr. Neuropharmacol. 8 2010 97 104
-
(2010)
Curr. Neuropharmacol.
, vol.8
, pp. 97-104
-
-
Buffoli, B.1
-
43
-
-
33947262188
-
Physiological evidence that pyramidal neurons lack functional water channels
-
R.D. Andrew, M.W. Labron, S.E. Boehnke, L. Carnduff, and S.A. Kirov Physiological evidence that pyramidal neurons lack functional water channels Cereb. Cortex 17 2007 787 802
-
(2007)
Cereb. Cortex
, vol.17
, pp. 787-802
-
-
Andrew, R.D.1
Labron, M.W.2
Boehnke, S.E.3
Carnduff, L.4
Kirov, S.A.5
-
44
-
-
80055082820
-
Glial-conditional deletion of aquaporin-4 (Aqp4) reduces blood-brain water uptake and confers barrier function on perivascular astrocyte endfeet
-
N.N. Haj-Yasein, G.F. Vindedal, M. Eilert-Olsen, G.A. Gunderson, O. Skare, P. Laake, A. Klungland, A.E. Thoren, J.M. Burkhardt, O.P. Ottersen, and E.A. Nagelhus Glial-conditional deletion of aquaporin-4 (Aqp4) reduces blood-brain water uptake and confers barrier function on perivascular astrocyte endfeet PNAS 108 2011 17815 17820
-
(2011)
PNAS
, vol.108
, pp. 17815-17820
-
-
Haj-Yasein, N.N.1
Vindedal, G.F.2
Eilert-Olsen, M.3
Gunderson, G.A.4
Skare, O.5
Laake, P.6
Klungland, A.7
Thoren, A.E.8
Burkhardt, J.M.9
Ottersen, O.P.10
Nagelhus, E.A.11
-
45
-
-
35348868575
-
Dynamic relationship between neurostimulation and N-acetylaspartate metabolism in the human visual cortex. Evidence that NAA functions as a molecular water pump during visual stimulation
-
M.H. Baslow, J. Hrabe, and D.N. Guilfoyle Dynamic relationship between neurostimulation and N-acetylaspartate metabolism in the human visual cortex. Evidence that NAA functions as a molecular water pump during visual stimulation J. Mol. Neurosci. 32 2007 235 245
-
(2007)
J. Mol. Neurosci.
, vol.32
, pp. 235-245
-
-
Baslow, M.H.1
Hrabe, J.2
Guilfoyle, D.N.3
-
46
-
-
84863872083
-
Stimulation-induced decreases in the diffusion of extra-vascular water in the human visual cortex: A window in time and space on mechanisms of brain water transport and economy
-
M.H. Baslow, C. Hu, and D.N. Guilfoyle Stimulation-induced decreases in the diffusion of extra-vascular water in the human visual cortex: a window in time and space on mechanisms of brain water transport and economy J. Mol. Neurosci. 47 2012 639 648
-
(2012)
J. Mol. Neurosci.
, vol.47
, pp. 639-648
-
-
Baslow, M.H.1
Hu, C.2
Guilfoyle, D.N.3
-
47
-
-
0035979210
-
Transient decrease in water diffusion observed in human occipital cortex during visual stimulation
-
A. Darquie, J.-B. Poline, C. Poupon, H. Saint-Jalmes, and D. Le Bihan Transient decrease in water diffusion observed in human occipital cortex during visual stimulation PNAS 98 2001 9391 9395
-
(2001)
PNAS
, vol.98
, pp. 9391-9395
-
-
Darquie, A.1
Poline, J.-B.2
Poupon, C.3
Saint-Jalmes, H.4
Le Bihan, D.5
-
48
-
-
33744501825
-
Direct and fast detection of neuronal activation in the human brain with diffusion MRI
-
D. Le Bihan, S. Urayama, A. Toshihiko, T. Hanakawa, and H. Fukuyama Direct and fast detection of neuronal activation in the human brain with diffusion MRI PNAS 103 2006 8263 8268
-
(2006)
PNAS
, vol.103
, pp. 8263-8268
-
-
Le Bihan, D.1
Urayama, S.2
Toshihiko, A.3
Hanakawa, T.4
Fukuyama, H.5
-
49
-
-
79954563176
-
Teaching NeuroImages: Honeycomb appearance of the brain in a patient with Canavan disease
-
S. Pradhan, and G. Goyal Teaching NeuroImages: honeycomb appearance of the brain in a patient with Canavan disease Neurology 76 2011 e68
-
(2011)
Neurology
, vol.76
, pp. 68
-
-
Pradhan, S.1
Goyal, G.2
-
50
-
-
33847141978
-
N-acetylaspartate in the CNS: From neurodiagnostics to neurobiology
-
J.R. Moffett, B. Ross, P. Arun, C.N. Madhavarao, and A.M. Namboodiri N-acetylaspartate in the CNS: from neurodiagnostics to neurobiology Prog. Neurobiol. 81 2007 89 131
-
(2007)
Prog. Neurobiol.
, vol.81
, pp. 89-131
-
-
Moffett, J.R.1
Ross, B.2
Arun, P.3
Madhavarao, C.N.4
Namboodiri, A.M.5
-
51
-
-
20144389547
-
Defective N-acetylaspartate catabolism reduces brain acetate levels and myelin lipid synthesis in Canavan's disease
-
C.N. Madhavarao, P. Arun, J.R. Moffett, S. Szucs, S. Surendran, R. Matalon, J. Garbern, D. Hristova, A. Johnson, W. Jiang, and M.A. Namboodiri Defective N-acetylaspartate catabolism reduces brain acetate levels and myelin lipid synthesis in Canavan's disease Proc. Natl. Acad. Sci. U. S. A. 102 2005 5221 5226
-
(2005)
Proc. Natl. Acad. Sci. U. S. A.
, vol.102
, pp. 5221-5226
-
-
Madhavarao, C.N.1
Arun, P.2
Moffett, J.R.3
Szucs, S.4
Surendran, S.5
Matalon, R.6
Garbern, J.7
Hristova, D.8
Johnson, A.9
Jiang, W.10
Namboodiri, M.A.11
-
52
-
-
77952240030
-
Methamphetamine-induced neuronal protein NAT8L is the biosynthetic enzyme: Implications for specialized acetyl coenzyme A metabolism in the CNS
-
P.S. Ariyannur, J.R. Moffett, P. Manickam, N. Pattabiraman, P. Arun, A. Nitta, T. Nabeshima, C.N. Madhavarao, and A.M.A. Namboodiri Methamphetamine- induced neuronal protein NAT8L is the biosynthetic enzyme: Implications for specialized acetyl coenzyme A metabolism in the CNS Brain Res. 1335 2010 1 13
-
(2010)
Brain Res.
, vol.1335
, pp. 1-13
-
-
Ariyannur, P.S.1
Moffett, J.R.2
Manickam, P.3
Pattabiraman, N.4
Arun, P.5
Nitta, A.6
Nabeshima, T.7
Madhavarao, C.N.8
Namboodiri, A.M.A.9
-
53
-
-
84865964454
-
Aspartoacylase supports oxidative energy metabolism during myelination
-
10.1038/jcbfm.2012.66
-
J.S. Francis, L. Strande, V. Markov, and P. Leone Aspartoacylase supports oxidative energy metabolism during myelination J. Cerebr. Blood Flow Metab. 23 May 2012 1 12 10.1038/jcbfm.2012.66
-
(2012)
J. Cerebr. Blood Flow Metab.
, pp. 1-12
-
-
Francis, J.S.1
Strande, L.2
Markov, V.3
Leone, P.4
-
54
-
-
0013957861
-
Acetyl metabolism in the nervous system. N-acetyl-l-aspartic acid and the biosynthesis of brain lipids
-
A.F. D'Adamo, and F.M. Yatsu Acetyl metabolism in the nervous system. N-acetyl-l-aspartic acid and the biosynthesis of brain lipids J. Neurochem. 13 1966 961 963
-
(1966)
J. Neurochem.
, vol.13
, pp. 961-963
-
-
D'Adamo, A.F.1
Yatsu, F.M.2
-
55
-
-
32044463622
-
Mild-onset presentation of Canvan's disease associated with novel G212A point mutation in aspartoacylase gene, 2006 Mild-onset presentation of Canvan's disease associated with novel G212A point mutation in aspartoacylase gene
-
C.G. Janson, E.H. Kolodny, B.-J. Zeng, S. Raghavan, G. Pastores, P. Torres, M. Assadi, S. McPhee, O. Goldfarb, B. Saslow, A. Freese, D.J. Wang, L. Bilaniuk, D. Shera, and P. Leone Mild-onset presentation of Canvan's disease associated with novel G212A point mutation in aspartoacylase gene, 2006 Mild-onset presentation of Canvan's disease associated with novel G212A point mutation in aspartoacylase gene Ann. Neurol. 59 2006 428 431
-
(2006)
Ann. Neurol.
, vol.59
, pp. 428-431
-
-
Janson, C.G.1
Kolodny, E.H.2
Zeng, B.-J.3
Raghavan, S.4
Pastores, G.5
Torres, P.6
Assadi, M.7
McPhee, S.8
Goldfarb, O.9
Saslow, B.10
Freese, A.11
Wang, D.J.12
Bilaniuk, L.13
Shera, D.14
Leone, P.15
-
56
-
-
38949128327
-
Homozygosity for mutation G212A of the gene for aspartoacylase is associated with atypical form of Canavan disease
-
M. Velinov, N. Zellers, J. Styles, and K. Wisniewski Homozygosity for mutation G212A of the gene for aspartoacylase is associated with atypical form of Canavan disease Clin. Genet. 73 2008 288 289
-
(2008)
Clin. Genet.
, vol.73
, pp. 288-289
-
-
Velinov, M.1
Zellers, N.2
Styles, J.3
Wisniewski, K.4
-
57
-
-
0035072775
-
Absence of N-acetylaspartate in the human brain: Impact on neurospectroscopy?
-
E. Martin, A. Capone, J. Schneider, J. Hennig, and T. Thorsten Absence of N-acetylaspartate in the human brain: impact on neurospectroscopy? Ann. Neurol. 49 2001 518 521
-
(2001)
Ann. Neurol.
, vol.49
, pp. 518-521
-
-
Martin, E.1
Capone, A.2
Schneider, J.3
Hennig, J.4
Thorsten, T.5
-
58
-
-
72449146905
-
Molecular identification of aspartate N-acetyltransferase and its mutation in hypoacetylaspartia
-
E. Wiame, D. Tyteca, N. Pierrot, F. Collard, M. Amyere, G. Noel, J. Desmedt, M.-C. Nassogne, M. Vikkula, J.-N. Octave, M.-F. Vincent, P.J. Courtoy, E. Boltshauser, and E. Van Shaftingen Molecular identification of aspartate N-acetyltransferase and its mutation in hypoacetylaspartia Biochem. J. 425 2010 127 136
-
(2010)
Biochem. J.
, vol.425
, pp. 127-136
-
-
Wiame, E.1
Tyteca, D.2
Pierrot, N.3
Collard, F.4
Amyere, M.5
Noel, G.6
Desmedt, J.7
Nassogne, M.-C.8
Vikkula, M.9
Octave, J.-N.10
Vincent, M.-F.11
Courtoy, P.J.12
Boltshauser, E.13
Van Shaftingen, E.14
-
59
-
-
77953230058
-
Metabolic acetate therapy improves phenotype in the tremor rat model of Canavan disease
-
P. Arun, C.N. Madhavarao, J.R. Moffett, K. Hamilton, N.E. Grunberg, P.S. Ariyannur, W.A. Gahl, Y. Anikster, S. Mog, W.C. Hallows, J.M. Denu, and A.M. Namboodiri Metabolic acetate therapy improves phenotype in the tremor rat model of Canavan disease J. Inherit. Metab. Dis. 33 2010 195 210
-
(2010)
J. Inherit. Metab. Dis.
, vol.33
, pp. 195-210
-
-
Arun, P.1
Madhavarao, C.N.2
Moffett, J.R.3
Hamilton, K.4
Grunberg, N.E.5
Ariyannur, P.S.6
Gahl, W.A.7
Anikster, Y.8
Mog, S.9
Hallows, W.C.10
Denu, J.M.11
Namboodiri, A.M.12
-
60
-
-
80053479492
-
Astrocytes are an early target in osmotic demyelination syndrome
-
F. Gankam-Kengne, C. Nicaise, A. Soupart, A. Boom, J. Schiettecatte, R. Pochet, J.P. Brion, and G. Decaux Astrocytes are an early target in osmotic demyelination syndrome J. Am. Soc. Nephrol. 22 2011 1834 1845
-
(2011)
J. Am. Soc. Nephrol.
, vol.22
, pp. 1834-1845
-
-
Gankam-Kengne, F.1
Nicaise, C.2
Soupart, A.3
Boom, A.4
Schiettecatte, J.5
Pochet, R.6
Brion, J.P.7
Decaux, G.8
-
61
-
-
79958041841
-
A safety trial of high dose glyceryl triacetate for Canavan disease
-
10.1016/j.ymgme.2011.03.012
-
R. Segel, Y. Anikster, S. Zevin, A. Steinberg, W.A. Gahl, D. Fisher, O. Staretz-Chacham, A. Zimran, and G. Altarescu A safety trial of high dose glyceryl triacetate for Canavan disease Mol. Genet. Metab. 2011 10.1016/j.ymgme.2011.03.012
-
(2011)
Mol. Genet. Metab.
-
-
Segel, R.1
Anikster, Y.2
Zevin, S.3
Steinberg, A.4
Gahl, W.A.5
Fisher, D.6
Staretz-Chacham, O.7
Zimran, A.8
Altarescu, G.9
-
62
-
-
0031934413
-
Diffusion theory, the cell and the synapse
-
D.N. Wheatley Diffusion theory, the cell and the synapse Biosystems 45 1998 151 163
-
(1998)
Biosystems
, vol.45
, pp. 151-163
-
-
Wheatley, D.N.1
-
63
-
-
80051728609
-
Molecular biology of the blood-brain and blood-cerebrospinal fluid barriers: Similarities and differences
-
10.1186/2045-8118-8-3
-
Z. Redzic Molecular biology of the blood-brain and blood-cerebrospinal fluid barriers: similarities and differences Fluids Barriers CNS 8 2011 10.1186/2045-8118-8-3
-
(2011)
Fluids Barriers CNS
, vol.8
-
-
Redzic, Z.1
-
64
-
-
0022494640
-
N-acetylaspartic aciduria in a child with a progressive cerebral atrophy
-
E.A. Kvittingen, G. Guldal, S. Borsting, I.O. Skalpe, O. Stokke, and E. Jellum N-acetylaspartic aciduria in a child with a progressive cerebral atrophy Clin. Chim. Acta 158 1986 217 227
-
(1986)
Clin. Chim. Acta
, vol.158
, pp. 217-227
-
-
Kvittingen, E.A.1
Guldal, G.2
Borsting, S.3
Skalpe, I.O.4
Stokke, O.5
Jellum, E.6
-
65
-
-
0013361563
-
N-acetylaspartylglutamate in Canavan disease: An adverse effector?
-
A.P. Burlina, V. Ferrari, P. Divry, W. Gradowska, C. Jakobs, M.J. Bennett, A.C. Sewell, C. Dionisi-Vici, and A.B. Burlina N- acetylaspartylglutamate in Canavan disease: an adverse effector? Eur. J. Pediatr. 158 1999 406 409
-
(1999)
Eur. J. Pediatr.
, vol.158
, pp. 406-409
-
-
Burlina, A.P.1
Ferrari, V.2
Divry, P.3
Gradowska, W.4
Jakobs, C.5
Bennett, M.J.6
Sewell, A.C.7
Dionisi-Vici, C.8
Burlina, A.B.9
-
66
-
-
84874647353
-
RAAV-mediated, systemically delivered and miRNA-regulated CNS gene transfer effectively correct metabolic and psychomotor defect, alleviate neuropathology, and prolong survival of ASPA -/- mice with Canavan disease
-
(conference abstract)
-
S. Ahmed, S. Szucs, H. Zhang, Q. Su, R. He, M. Sena-Esteves, T. Flotte, R. Matalon, and G. Gao rAAV-mediated, systemically delivered and miRNA-regulated CNS gene transfer effectively correct metabolic and psychomotor defect, alleviate neuropathology, and prolong survival of ASPA -/- mice with Canavan disease Mol. Ther. 19 2011 S249 S250 (conference abstract)
-
(2011)
Mol. Ther.
, vol.19
-
-
Ahmed, S.1
Szucs, S.2
Zhang, H.3
Su, Q.4
He, R.5
Sena-Esteves, M.6
Flotte, T.7
Matalon, R.8
Gao, G.9
-
67
-
-
84874650818
-
Successful in vivo gene therapy for a white matter disease after adeno-associated virus vector delivery to oligodendrocytes
-
424-425 (conference abstract)
-
N. Mersmann, E.-M. Kramer-Albers, O. Teahan, and M. Klugmann Successful in vivo gene therapy for a white matter disease after adeno-associated virus vector delivery to oligodendrocytes J. Gene Med. 13 2011 7 8 424-425 (conference abstract)
-
(2011)
J. Gene Med.
, vol.13
, pp. 7-8
-
-
Mersmann, N.1
Kramer-Albers, E.-M.2
Teahan, O.3
Klugmann, M.4
-
68
-
-
84862760065
-
Nutritional and medicinal aspects of d-amino acids
-
M. Friedman, and C.E. Levin Nutritional and medicinal aspects of d-amino acids Amino Acids 42 2012 1553 1582
-
(2012)
Amino Acids
, vol.42
, pp. 1553-1582
-
-
Friedman, M.1
Levin, C.E.2
-
69
-
-
0036014995
-
The effects of lithium chloride and other substances on levels of brain N-acetyl-l-aspartic acid in Canavan disease-like rats
-
M.H. Baslow, K. Kitada, R.F. Suckow, B.L. Hungund, and T. Serikawa The effects of lithium chloride and other substances on levels of brain N-acetyl-l-aspartic acid in Canavan disease-like rats Neurochemical. Res. 27 2002 403 406
-
(2002)
Neurochemical. Res.
, vol.27
, pp. 403-406
-
-
Baslow, M.H.1
Kitada, K.2
Suckow, R.F.3
Hungund, B.L.4
Serikawa, T.5
-
70
-
-
25644456835
-
Lithium citrate for Canavan disease
-
C.G. Janson, M. Assadi, J. Francis, L. Bilaniuk, D. Shera, and P. Leone Lithium citrate for Canavan disease Pediatr. Neurol. 33 2005 235 243
-
(2005)
Pediatr. Neurol.
, vol.33
, pp. 235-243
-
-
Janson, C.G.1
Assadi, M.2
Francis, J.3
Bilaniuk, L.4
Shera, D.5
Leone, P.6
-
72
-
-
78651419742
-
Modification of aspartoacylase for potential use in enzyme replacement therapy for the treatment of Canavan disease
-
S. Zano, R. Malik, S. Szucs, R. Matalon, and R.E. Viola Modification of aspartoacylase for potential use in enzyme replacement therapy for the treatment of Canavan disease Mol. Genet. Metab. 102 2011 176 180
-
(2011)
Mol. Genet. Metab.
, vol.102
, pp. 176-180
-
-
Zano, S.1
Malik, R.2
Szucs, S.3
Matalon, R.4
Viola, R.E.5
-
73
-
-
0033736818
-
Effects of ethanol and of alcohol dehydrogenase inhibitors on the reduction of N-acetylaspartate levels of brain in mice in vivo. A search for substances that may have therapeutic value in the treatment of Canavan disease, an autosomally inherited recessive CNS disease
-
M.H. Baslow, R.F. Suckow, and B.L. Hungund Effects of ethanol and of alcohol dehydrogenase inhibitors on the reduction of N-acetylaspartate levels of brain in mice in vivo. A search for substances that may have therapeutic value in the treatment of Canavan disease, an autosomally inherited recessive CNS disease J. Inherit. Metab. Dis. 23 2000 684 692
-
(2000)
J. Inherit. Metab. Dis.
, vol.23
, pp. 684-692
-
-
Baslow, M.H.1
Suckow, R.F.2
Hungund, B.L.3
-
74
-
-
84855421463
-
Intravenous ethanol infusion decreases human cortical γ-aminobutyric acid and N-acetylaspartate as measured with proton magnetic resonance spectroscopy at 4 Tesla
-
R. Gomez, K.L. Behar, J. Watzl, S.A. Weinzimer, B. Gulanski, G. Sanacora, J. Koretski, E. Guidone, L. Jiang, I.L. Petrakis, B. Pittman, J.H. Krystal, and G.F. Mason Intravenous ethanol infusion decreases human cortical γ-aminobutyric acid and N-acetylaspartate as measured with proton magnetic resonance spectroscopy at 4 Tesla Biol. Psychiatry 71 2011 239 246
-
(2011)
Biol. Psychiatry
, vol.71
, pp. 239-246
-
-
Gomez, R.1
Behar, K.L.2
Watzl, J.3
Weinzimer, S.A.4
Gulanski, B.5
Sanacora, G.6
Koretski, J.7
Guidone, E.8
Jiang, L.9
Petrakis, I.L.10
Pittman, B.11
Krystal, J.H.12
Mason, G.F.13
-
75
-
-
77952101691
-
Riluzole decreases synthesis of N-acetylaspartate and N-acetylaspartylglutamate in SH-SY5Y human neuroblastoma cells
-
P. Arun, J.R. Moffett, and A.M. Namboodiri Riluzole decreases synthesis of N-acetylaspartate and N-acetylaspartylglutamate in SH-SY5Y human neuroblastoma cells Brain Res. 1334 2010 25 30
-
(2010)
Brain Res.
, vol.1334
, pp. 25-30
-
-
Arun, P.1
Moffett, J.R.2
Namboodiri, A.M.3
-
76
-
-
0001328357
-
The spongy type of diffuse sclerosis
-
M. Wolman The spongy type of diffuse sclerosis Brain 81 1958 243 247
-
(1958)
Brain
, vol.81
, pp. 243-247
-
-
Wolman, M.1
-
77
-
-
0015292217
-
Cerebral spongy degeneration of infancy
-
B.T. Adornato, J.S. O'Brien, P.W. Lampert, T.F. Roe, and H.B. Neustein Cerebral spongy degeneration of infancy Neurology 22 1972 202 210
-
(1972)
Neurology
, vol.22
, pp. 202-210
-
-
Adornato, B.T.1
O'Brien, J.S.2
Lampert, P.W.3
Roe, T.F.4
Neustein, H.B.5
-
78
-
-
0015815575
-
Spongy degeneration of the central nervous system (Van Bogaert and Bertrand type; Canavan's disease)
-
M. Adachi, L. Schneck, J. Cara, and B.W. Volk Spongy degeneration of the central nervous system (Van Bogaert and Bertrand type; Canavan's disease) Hum. Pathol. 4 1973 331 347
-
(1973)
Hum. Pathol.
, vol.4
, pp. 331-347
-
-
Adachi, M.1
Schneck, L.2
Cara, J.3
Volk, B.W.4
-
79
-
-
0023818297
-
Aspartoacylase deficiency and N-acetylaspartic aciduria in patients with Canavan disease
-
R. Matalon, K. Michals, D. Sebesta, M. Deanching, P. Gashkoff, and J. Casanova Aspartoacylase deficiency and N-acetylaspartic aciduria in patients with Canavan disease Amer. J. Med. Genet. 29 1988 463 471
-
(1988)
Amer. J. Med. Genet.
, vol.29
, pp. 463-471
-
-
Matalon, R.1
Michals, K.2
Sebesta, D.3
Deanching, M.4
Gashkoff, P.5
Casanova, J.6
-
80
-
-
54349116829
-
N-acetylglutamate and N-acetylaspartate in soybeans (Glycine max L.) maize (Zea maize L.), and other foodstuffs
-
A.O. Hession, E.G. Esrey, R.A. Croes, and C.A. Maxwell N-acetylglutamate and N-acetylaspartate in soybeans (Glycine max L.) maize (Zea maize L.), and other foodstuffs J. Agric. Food Chem. 56 2008 9121 9126
-
(2008)
J. Agric. Food Chem.
, vol.56
, pp. 9121-9126
-
-
Hession, A.O.1
Esrey, E.G.2
Croes, R.A.3
Maxwell, C.A.4
-
81
-
-
0013919162
-
Metabolism of N-acetyl-l-aspartic acid in mice
-
L. Berlinguet, and M. Laliberte Metabolism of N-acetyl-l-aspartic acid in mice Can. J. Biochem. 44 1966 783 789
-
(1966)
Can. J. Biochem.
, vol.44
, pp. 783-789
-
-
Berlinguet, L.1
Laliberte, M.2
-
82
-
-
80054873256
-
Two-generation reproductive and developmental toxicity assessment of dietary N-acetyl-l-aspartic acid in rats
-
S. Karaman, J. Barnett Jr., G.P. Sykes, B. Hong, and B. Delaney Two-generation reproductive and developmental toxicity assessment of dietary N-acetyl-l-aspartic acid in rats Food Chem. Toxicol. 49 2011 3192 3205
-
(2011)
Food Chem. Toxicol.
, vol.49
, pp. 3192-3205
-
-
Karaman, S.1
Barnett, Jr.J.2
Sykes, G.P.3
Hong, B.4
Delaney, B.5
|