-
1
-
-
0022938807
-
Mitochondrial benzodiazepine receptors as potential modulators of intermediary metabolism
-
Anholt, R.R.H. (1986). Mitochondrial benzodiazepine receptors as potential modulators of intermediary metabolism. TiPS 7:506-511.
-
(1986)
TiPS
, vol.7
, pp. 506-511
-
-
Anholt, R.R.H.1
-
2
-
-
0023943181
-
Differential alterations of various cholinergic markers in cortical and subcortical regions of human brain in Alzheimer's disease
-
Arauja, D.M., Lapchak, P.A., Robitaille, Y., Gauthier, S. and Quirion, R. (1988). Differential alterations of various cholinergic markers in cortical and subcortical regions of human brain in Alzheimer's disease. J. Neurochem. 50:19114-1923.
-
(1988)
J. Neurochem.
, vol.50
, pp. 19114-21923
-
-
Arauja, D.M.1
Lapchak, P.A.2
Robitaille, Y.3
Gauthier, S.4
Quirion, R.5
-
3
-
-
0021235972
-
Increase of tryptophan and 5-hydroxyindoleacetic acid in the brain of ornithine carbamoyl transferase deficient sparse-fur mice
-
Bachmann, C. and Colombo, J.P. (1984). Increase of tryptophan and 5-hydroxyindoleacetic acid in the brain of ornithine carbamoyl transferase deficient sparse-fur mice. Pediatr. Res. 18:372-375.
-
(1984)
Pediatr. Res.
, vol.18
, pp. 372-375
-
-
Bachmann, C.1
Colombo, J.P.2
-
4
-
-
0025763082
-
An investigation of the mechanisms of delayed neuron degeneration caused by direct injection of quinolinic acid into the rat striatum in vivo
-
Bakker, M.H.M. and Foster, A.C. (1991). An investigation of the mechanisms of delayed neuron degeneration caused by direct injection of quinolinic acid into the rat striatum in vivo. Neuroscience 42:387-395.
-
(1991)
Neuroscience
, vol.42
, pp. 387-395
-
-
Bakker, M.H.M.1
Foster, A.C.2
-
5
-
-
0012504864
-
L-acetyl-carnitine in Alzheimer Disease (AD) and Senile Dementia of the Alzheimer Type (SDAT)
-
A. Agnoli, J. Cahn, N. Larsen and R. Mayeux, eds., John Libbey Eurotext, Paris
-
Bassi S., Ferrarese, C., Finola, M.G., Frattola, L., Iannucelli, M., Meregalli, S. et al. (1988). L-acetyl-carnitine in Alzheimer Disease (AD) and Senile Dementia of the Alzheimer Type (SDAT), in Senile Dementias (Second International Symposium) (A. Agnoli, J. Cahn, N. Larsen and R. Mayeux, eds.), John Libbey Eurotext, Paris, pp. 461-466.
-
(1988)
Senile Dementias (Second International Symposium)
, pp. 461-466
-
-
Bassi, S.1
Ferrarese, C.2
Finola, M.G.3
Frattola, L.4
Iannucelli, M.5
Meregalli, S.6
-
6
-
-
0027383086
-
Quinolinic acid in children with congenital hyperammonemia
-
Batshaw, M.L., Robinson, M.B., Hyland, K., Djali, S. and Heyes, M.P. (1993). Quinolinic acid in children with congenital hyperammonemia. Ann. Neurol. 34:676-681.
-
(1993)
Ann. Neurol.
, vol.34
, pp. 676-681
-
-
Batshaw, M.L.1
Robinson, M.B.2
Hyland, K.3
Djali, S.4
Heyes, M.P.5
-
7
-
-
0023932115
-
Effect of sodium benzoate and sodium phenylacetate on brain serotonin turnover in the ornithine transcarbamylase-deficient sparse-fur mouse
-
Batshaw, , ML., Hyman, S.L., Coyle, J.T., Robinson, M.B., Qureshi, I.A., Millits, E.D., and Quaskey, S. (1988). Effect of sodium benzoate and sodium phenylacetate on brain serotonin turnover in the ornithine transcarbamylase-deficient sparse-fur mouse. Pediatr. Res. 23:368-374.
-
(1988)
Pediatr. Res.
, vol.23
, pp. 368-374
-
-
Batshaw, M.L.1
Hyman, S.L.2
Coyle, J.T.3
Robinson, M.B.4
Qureshi, I.A.5
Millits, E.D.6
Quaskey, S.7
-
8
-
-
0025004380
-
Application of reduced-size liver transplants as split grafts, auxiliary orthotopic grafts, and liver related segmental transplants
-
Broelsch, E.C., Emond, J.C., Whitington, P.F., Thistlethwaite, J.R., Baker, A.L., and Lichtor, J.L. (1990). Application of reduced-size liver transplants as split grafts, auxiliary orthotopic grafts, and liver related segmental transplants. Ann. Surg. 212:368-375.
-
(1990)
Ann. Surg.
, vol.212
, pp. 368-375
-
-
Broelsch, E.C.1
Emond, J.C.2
Whitington, P.F.3
Thistlethwaite, J.R.4
Baker, A.L.5
Lichtor, J.L.6
-
9
-
-
0001034416
-
Urea cycle enzymes.
-
C.R. Scriver, A.L. Beaudet, W.S. Sly et al. (eds.) New York: McGraw-Hill Information Services
-
Brusilow, S.W. and Horwich, A.L. (1995). Urea cycle enzymes. In (C.R. Scriver, A.L. Beaudet, W.S. Sly et al. (eds.) The Metabolic and Molecular Basis of Inherited Disease, 7th Ed. New York: McGraw-Hill Information Services, pp. 1187-1232.
-
(1995)
The Metabolic and Molecular Basis of Inherited Disease, 7th Ed.
, pp. 1187-1232
-
-
Brusilow, S.W.1
Horwich, A.L.2
-
10
-
-
0018597819
-
New pathways of nitrogen excretion in inborn errors of urea synthesis
-
Brusilow, S.W., Valle, D.L., and Batshaw, M. (1979). New pathways of nitrogen excretion in inborn errors of urea synthesis. Lancet 2:452-454.
-
(1979)
Lancet
, vol.2
, pp. 452-454
-
-
Brusilow, S.W.1
Valle, D.L.2
Batshaw, M.3
-
11
-
-
0021916126
-
Tryptophan and serotonin turnover rate in the brain of genetically hyperammonemic mice
-
Chaouloff, F., Laude, D., Mignot, E., Kamoun, P., and Elghozi, J.L. (1985). Tryptophan and serotonin turnover rate in the brain of genetically hyperammonemic mice. Neurochem. Int. 7:143-153.
-
(1985)
Neurochem. Int.
, vol.7
, pp. 143-153
-
-
Chaouloff, F.1
Laude, D.2
Mignot, E.3
Kamoun, P.4
Elghozi, J.L.5
-
12
-
-
0027464868
-
Ornithine transcarbamylase deficiency presenting with stroke-like episodes
-
Christodoulou, J., Qureshi, I.A., McInnes, R.F. and Clarke, J.T.R. (1993). Ornithine transcarbamylase deficiency presenting with stroke-like episodes. J. Pediatr. 122:423-425.
-
(1993)
J. Pediatr.
, vol.122
, pp. 423-425
-
-
Christodoulou, J.1
Qureshi, I.A.2
McInnes, R.F.3
Clarke, J.T.R.4
-
13
-
-
0015177260
-
Congenital disorders of the urea cycle and ammonia detoxification
-
Karger. Basel
-
Colombo, J.P. (1971). Congenital disorders of the urea cycle and ammonia detoxification. Monogr. Paediatr. 1, Karger. Basel.
-
(1971)
Monogr. Paediatr.
, vol.1
-
-
Colombo, J.P.1
-
14
-
-
0027498888
-
Magnetic resonance spectroscopy shows increased brain glutamine in ornithine carbamoyl transferase deficiency
-
Connelly, A., Cross, J.H., Gadian, D.G., Hunter, J.V., Kirkham, F.J., and Leonard, J.V. (1993). Magnetic resonance spectroscopy shows increased brain glutamine in ornithine carbamoyl transferase deficiency. Pediatr. Res. 33:77-81.
-
(1993)
Pediatr. Res.
, vol.33
, pp. 77-81
-
-
Connelly, A.1
Cross, J.H.2
Gadian, D.G.3
Hunter, J.V.4
Kirkham, F.J.5
Leonard, J.V.6
-
15
-
-
0042461295
-
Abnormal ornithine carbamyl transferase in mice having the sparse-fur mutation
-
Demars, R., Levan, S.L., Trend, B.L., and Russel, L.B. (1976). Abnormal ornithine carbamyl transferase in mice having the sparse-fur mutation. Proc. Natl. Acad. Sci. USA 23:1693-1698.
-
(1976)
Proc. Natl. Acad. Sci. USA
, vol.23
, pp. 1693-1698
-
-
Demars, R.1
Levan, S.L.2
Trend, B.L.3
Russel, L.B.4
-
16
-
-
0023945506
-
Severe cerebral damage in ornithine transcarbamylase deficiency
-
Dolman, C.L. Clasen, R.A., and Dorovini-Zis K. (1988). Severe cerebral damage in ornithine transcarbamylase deficiency. Clin. Neuropathol. 7:10-15.
-
(1988)
Clin. Neuropathol.
, vol.7
, pp. 10-15
-
-
Dolman, C.L.1
Clasen, R.A.2
Dorovini-Zis, K.3
-
17
-
-
0022619794
-
Ornithine transcarbamylase deficiency: Neuropathologic changes acquired in utero
-
Filloux, F., Townsend, J.J., and Leonard, C. (1986). Ornithine transcarbamylase deficiency: neuropathologic changes acquired in utero. J. Pediat. 108:942-945.
-
(1986)
J. Pediat.
, vol.108
, pp. 942-945
-
-
Filloux, F.1
Townsend, J.J.2
Leonard, C.3
-
19
-
-
0021367838
-
Ornithine transcarbamylase deficiency: Neuropathological study
-
Harding, B.N., Leonard, J.V., and Erdohazi, M. (1984). Ornithine transcarbamylase deficiency: neuropathological study. Eur. J. Pediatr. 141:215.
-
(1984)
Eur. J. Pediatr.
, vol.141
, pp. 215
-
-
Harding, B.N.1
Leonard, J.V.2
Erdohazi, M.3
-
20
-
-
0001904134
-
Nutritional and metabolic disorders
-
D.I. Graham and P.L. Lantos, eds Arnold, London, UK, 1997
-
Harper, C.G. and Butterworth, R.F. (1997). Nutritional and metabolic disorders in. (D.I. Graham and P.L. Lantos, eds) Greenfield's Neuropathology, Arnold, London, UK, 1997, pp. 601-655.
-
(1997)
Greenfield's Neuropathology
, pp. 601-655
-
-
Harper, C.G.1
Butterworth, R.F.2
-
21
-
-
0029012513
-
Orthotopic liver transplantation for ornithine transcarbamylase deficiency with hyperammonemic encephalopathy
-
Hasegawa, T., Tzakis, A.G., Todo, S., Reyes, J., Nour, B., Finegold, D.N. et al. (1995). Orthotopic liver transplantation for ornithine transcarbamylase deficiency with hyperammonemic encephalopathy. J. Pediat. Surg. 30:863-865.
-
(1995)
J. Pediat. Surg.
, vol.30
, pp. 863-865
-
-
Hasegawa, T.1
Tzakis, A.G.2
Todo, S.3
Reyes, J.4
Nour, B.5
Finegold, D.N.6
-
22
-
-
0023616487
-
Behavioral management of feeding disturbances in urea cycle and organic acid disorders
-
Hyman. S.L., Porter, C.A., Page, J.J., Iwata, B.A., Kissel, R., and Batshaw, M.L. (1988). Behavioral management of feeding disturbances in urea cycle and organic acid disorders. J. Pediatr. 111:558-562.
-
(1988)
J. Pediatr.
, vol.111
, pp. 558-562
-
-
Hyman, S.L.1
Porter, C.A.2
Page, J.J.3
Iwata, B.A.4
Kissel, R.5
Batshaw, M.L.6
-
23
-
-
0024832270
-
Serotonin- And catecholamine-related substances in the brain of ornithine transcarbamylase-deficient sparse-fur mice in the hyperammonemic state: Comparison of two procedures for obtaining brain extract, decapitation and microwave irradiation
-
Inoue, I., Shimizu, T., Saheki, T., Noda, T., and Fukuda, T. (1989). Serotonin- and catecholamine-related substances in the brain of ornithine transcarbamylase-deficient sparse-fur mice in the hyperammonemic state: comparison of two procedures for obtaining brain extract, decapitation and microwave irradiation. Biochem. Med. Metab. Biol. 42:232-239.
-
(1989)
Biochem. Med. Metab. Biol.
, vol.42
, pp. 232-239
-
-
Inoue, I.1
Shimizu, T.2
Saheki, T.3
Noda, T.4
Fukuda, T.5
-
24
-
-
0029743996
-
Histological improvement in native liver after auxiliary partial liver transplantation for ornithine transcarbamylase deficiency
-
Inui, A., Fujisawa, T., Komatsu, H., Tanaka, K., and Inui, M. (1996). Histological improvement in native liver after auxiliary partial liver transplantation for ornithine transcarbamylase deficiency [letter]. Lancet 348:751-752.
-
(1996)
Lancet
, vol.348
, pp. 751-752
-
-
Inui, A.1
Fujisawa, T.2
Komatsu, H.3
Tanaka, K.4
Inui, M.5
-
25
-
-
0023689953
-
Sodium benzoate inhibits fatty acid oxidation in rat liver. Effect on ammonia levels
-
Kalbag, S.S. and Palekar, A.G. (1988). Sodium benzoate inhibits fatty acid oxidation in rat liver. Effect on ammonia levels. Biochem. Med. Metab. Biol. 40:133-142.
-
(1988)
Biochem. Med. Metab. Biol.
, vol.40
, pp. 133-142
-
-
Kalbag, S.S.1
Palekar, A.G.2
-
26
-
-
0020662172
-
Neurological features and computed tomography of the brain in children with ornithine carbamoyl transferase deficiency
-
Kendall, B.E., Kingsley, D.P.E., Leonard, J.V., Lingam, S., and Oberholzer, V.G. (1983). Neurological features and computed tomography of the brain in children with ornithine carbamoyl transferase deficiency. J. Neurol. Neurosurg. Psychiatry 46:28-34.
-
(1983)
J. Neurol. Neurosurg. Psychiatry
, vol.46
, pp. 28-34
-
-
Kendall, B.E.1
Kingsley, D.P.E.2
Leonard, J.V.3
Lingam, S.4
Oberholzer, V.G.5
-
27
-
-
0022972426
-
Brain α-ketoglutarate dehydrogenase complex: Kinetic properties, regional distribution and effects of inhibitors
-
Lai, J.C.K. and Cooper, A.J.L. (1986). Brain α-ketoglutarate dehydrogenase complex: kinetic properties, regional distribution and effects of inhibitors. J Neurochem. 47:1376-1386.
-
(1986)
J Neurochem.
, vol.47
, pp. 1376-1386
-
-
Lai, J.C.K.1
Cooper, A.J.L.2
-
28
-
-
0025298785
-
Increased densities of "peripheral-type" benzodiazepine receptors in autopsied brain tissue from cirrhotic patients with hepatic encephalopathy
-
Lavoie, J., Pomier Layrargues, G., and Butterworth, R.F. (1990). Increased densities of "peripheral-type" benzodiazepine receptors in autopsied brain tissue from cirrhotic patients with hepatic encephalopathy Hepatology 11:874-878.
-
(1990)
Hepatology
, vol.11
, pp. 874-878
-
-
Lavoie, J.1
Pomier Layrargues, G.2
Butterworth, R.F.3
-
29
-
-
0026332787
-
Suppression of neurotoxicity of ammonia by L-carnitine
-
Matsuoka, M., Igisu, H., Kohriyama, K., and Inoue, N. (1991). Suppression of neurotoxicity of ammonia by L-carnitine. Brain Res. 567:328-331.
-
(1991)
Brain Res.
, vol.567
, pp. 328-331
-
-
Matsuoka, M.1
Igisu, H.2
Kohriyama, K.3
Inoue, N.4
-
30
-
-
0027483623
-
Effects of L- And D-carnitine on brain energy metabolites in mice given sublethal doses of ammonium acetate
-
Matsuoka, M. and Igisu, H. (1993). Effects of L- and D-carnitine on brain energy metabolites in mice given sublethal doses of ammonium acetate. Pharmacol. & Toxicol. 72:145-147.
-
(1993)
Pharmacol. & Toxicol.
, vol.72
, pp. 145-147
-
-
Matsuoka, M.1
Igisu, H.2
-
31
-
-
0029045140
-
Free and esterified coenzyme A in the liver and muscles of chronically hyperammonemic mice treated with sodium benzoate
-
Michalak, A. and Qureshi, I.A. (1995a). Free and esterified coenzyme A in the liver and muscles of chronically hyperammonemic mice treated with sodium benzoate. Biochem. and Molec. Med. 54:96-104.
-
(1995)
Biochem. and Molec. Med.
, vol.54
, pp. 96-104
-
-
Michalak, A.1
Qureshi, I.A.2
-
32
-
-
0028849663
-
Tissue acylcarnitine and acyl-coenzyme A profiles in chronically hyperammonemic mice treated with sodium benzoate and supplementary L-carnitine
-
Michalak, A. and Qureshi, I.A. (1995b). Tissue acylcarnitine and acyl-coenzyme A profiles in chronically hyperammonemic mice treated with sodium benzoate and supplementary L-carnitine. Biomed. & Pharmacother. 49:350-357.
-
(1995)
Biomed. & Pharmacother.
, vol.49
, pp. 350-357
-
-
Michalak, A.1
Qureshi, I.A.2
-
33
-
-
0028567119
-
Ornithine transcarbamylase deficiency: A model for gene therapy.
-
V. Felipo and S. Grisolia, eds. Plenum Press, New York
-
Morsy, M.A. and Caskey, C.T. (1994). Ornithine transcarbamylase deficiency: a model for gene therapy. In (V. Felipo and S. Grisolia, eds.) Hepatic Encephalopathy, Hyperammonemia and Ammonia Toxicity, Plenum Press, New York, pp. 145-154.
-
(1994)
Hepatic Encephalopathy, Hyperammonemia and Ammonia Toxicity
, pp. 145-154
-
-
Morsy, M.A.1
Caskey, C.T.2
-
34
-
-
0021187612
-
Neurologic outcome in children with inborn errors of urea synthesis
-
Msall, M., Batshaw, M.L., Suss, R., Brusilow, S.W., and Mellits, E.D. (1984). Neurologic outcome in children with inborn errors of urea synthesis. New Engl. J. Med. 310:1500-1505.
-
(1984)
New Engl. J. Med.
, vol.310
, pp. 1500-1505
-
-
Msall, M.1
Batshaw, M.L.2
Suss, R.3
Brusilow, S.W.4
Mellits, E.D.5
-
35
-
-
0022450248
-
Influence of the route of administration on the protective effect of L-carnitine on acute hyperammonemia
-
O'Connor, J.E., Costell, M., Miguez, M.P., and Grisolia, S. (1986). Influence of the route of administration on the protective effect of L-carnitine on acute hyperammonemia. Biochem. Pharmacol. 35:3173-3176.
-
(1986)
Biochem. Pharmacol.
, vol.35
, pp. 3173-3176
-
-
O'Connor, J.E.1
Costell, M.2
Miguez, M.P.3
Grisolia, S.4
-
36
-
-
0042962457
-
Animal models of hereditary hyperammonemias.
-
A. Boulton, G. Baker, and R. Butterworth, eds. Humana Press, Clifton N.J.
-
Qureshi, I.A. (1992). Animal models of hereditary hyperammonemias. In: (A. Boulton, G. Baker, and R. Butterworth, eds.) Neuromethods, Vol. 22: Animal Models of Neurological Disease, II, Humana Press, Clifton N.J. pp. 329-356.
-
(1992)
Neuromethods, Vol. 22: Animal Models of Neurological Disease, II
, vol.22
, pp. 329-356
-
-
Qureshi, I.A.1
-
38
-
-
0028213411
-
Activities of monoamine oxidase-A and -B are altered in the brains of congenitally hyperammonemic sparse-fur (spf) mice
-
Raghavendra Rao, V.L., Qureshi, I.A., and Butterworth, R.F. (1994). Activities of monoamine oxidase-A and -B are altered in the brains of congenitally hyperammonemic sparse-fur (spf) mice. Neurosci. Lett. 170:27-30.
-
(1994)
Neurosci. Lett.
, vol.170
, pp. 27-30
-
-
Raghavendra Rao, V.L.1
Qureshi, I.A.2
Butterworth, R.F.3
-
39
-
-
0026752613
-
Effects of congenital hyperammonemia on the cerebral and hepatic levels of the intermediates of energy metabolism in spf mice
-
Ratnakumari, L., Qureshi, I.A., and Butterworth, R.F. (1992). Effects of congenital hyperammonemia on the cerebral and hepatic levels of the intermediates of energy metabolism in spf mice. Biochem. Biophys. Res. Commun. 184:746-751.
-
(1992)
Biochem. Biophys. Res. Commun.
, vol.184
, pp. 746-751
-
-
Ratnakumari, L.1
Qureshi, I.A.2
Butterworth, R.F.3
-
40
-
-
26844553499
-
Evidence for a severe cholinergic neuronal deficit in brain in congenital ornithine transcarbamylase (OTC) deficiency
-
Ratnakumari, L., Qureshi, I.A., and Butterworth, R.F. (1993a). Evidence for a severe cholinergic neuronal deficit in brain in congenital ornithine transcarbamylase (OTC) deficiency. Soc. Neurosci. Abs. 19:122.
-
(1993)
Soc. Neurosci. Abs.
, vol.19
, pp. 122
-
-
Ratnakumari, L.1
Qureshi, I.A.2
Butterworth, R.F.3
-
41
-
-
0027528327
-
Effect of sodium benzoate on cerebral and hepatic energy metabolites in spf mice with congenital hyperammonemia
-
Ratnakumari, L., Qureshi, I.A., and Butterworth, R.F. (1993b). Effect of sodium benzoate on cerebral and hepatic energy metabolites in spf mice with congenital hyperammonemia. Biochem. Pharmacol. 45:137-146.
-
(1993)
Biochem. Pharmacol.
, vol.45
, pp. 137-146
-
-
Ratnakumari, L.1
Qureshi, I.A.2
Butterworth, R.F.3
-
42
-
-
0027203913
-
Effect of L-carnitine on cerebral and hepatic energy metabolites in congenitally hyperammonemic sparse-fuce mice and its role during benzoate therapy
-
Ratnakumari, L., Qureshi, I.A., and Butterworth, R.F. (1993c). Effect of L-carnitine on cerebral and hepatic energy metabolites in congenitally hyperammonemic sparse-fuce mice and its role during benzoate therapy. Metabolism 42:1039-1046.
-
(1993)
Metabolism
, vol.42
, pp. 1039-1046
-
-
Ratnakumari, L.1
Qureshi, I.A.2
Butterworth, R.F.3
-
43
-
-
0028240536
-
Regional amino acid neurotransmitter changes in brains of spf/Y mice with congenital ornithine transcarbamylase deficiency
-
Ratnakumari, L., Qureshi, I.A., and Butterworth, R.F. (1994a). Regional amino acid neurotransmitter changes in brains of spf/Y mice with congenital ornithine transcarbamylase deficiency. Metab. Brain Dis. 9:43-51.
-
(1994)
Metab. Brain Dis.
, vol.9
, pp. 43-51
-
-
Ratnakumari, L.1
Qureshi, I.A.2
Butterworth, R.F.3
-
44
-
-
0027999155
-
Evidence for cholinergic neuronal loss in brain in congenital ornithine transcarbamylase deficiency
-
Ratnakumari, L., Qureshi, I.A., and Butterworth, R.F. (1994b). Evidence for cholinergic neuronal loss in brain in congenital ornithine transcarbamylase deficiency. Neurosci. Lett. 178:63-65.
-
(1994)
Neurosci. Lett.
, vol.178
, pp. 63-65
-
-
Ratnakumari, L.1
Qureshi, I.A.2
Butterworth, R.F.3
-
45
-
-
0029042318
-
Developmental deficiency of the cholinergic system in congenitally hyperammonemic spf mice: Effect of acetyl-L-carnitine
-
Ratnakumari, L., Qureshi, I.A., Maysinger, D., and Butterworth, R.F. (1995a). Developmental deficiency of the cholinergic system in congenitally hyperammonemic spf mice: effect of acetyl-L-carnitine. J Pharmacol. Exp. Ther. 274:437-443.
-
(1995)
J Pharmacol. Exp. Ther.
, vol.274
, pp. 437-443
-
-
Ratnakumari, L.1
Qureshi, I.A.2
Maysinger, D.3
Butterworth, R.F.4
-
47
-
-
3643092360
-
Arginine-related guanidino compounds and nitric oxide synthase in the brain of ornithine transcarbamylase deficient spf mutant mouse: Effect of metabolic arginine deficiency.
-
P.P. De Deyn et al., eds. John Libby and Co.
-
Ratnakumari, L., Qureshi, I.A., Butterworth, R.F., Marescau, B., and De Deyn, P.P. (1996a). Arginine-related guanidino compounds and nitric oxide synthase in the brain of ornithine transcarbamylase deficient spf mutant mouse: effect of metabolic arginine deficiency. In (P.P. De Deyn et al., eds.) Guanidino Compounds, John Libby and Co., pp. 17-20.
-
(1996)
Guanidino Compounds
, pp. 17-20
-
-
Ratnakumari, L.1
Qureshi, I.A.2
Butterworth, R.F.3
Marescau, B.4
De Deyn, P.P.5
-
49
-
-
0028892620
-
Evidence of excitotoxicity in the brain of the ornithine carbamoyltransferase deficient sparse fur mouse
-
Robinson, M.B., Hopkins, K., Batshaw, M.L., McLaughlin, B.A., Heyes, M.P., and Oster-Granite, M.L. (1995a). Evidence of excitotoxicity in the brain of the ornithine carbamoyltransferase deficient sparse fur mouse. Dev. Brain Res. 90:35-44.
-
(1995)
Dev. Brain Res.
, vol.90
, pp. 35-44
-
-
Robinson, M.B.1
Hopkins, K.2
Batshaw, M.L.3
McLaughlin, B.A.4
Heyes, M.P.5
Oster-Granite, M.L.6
-
50
-
-
84989718538
-
Prospects for gene therapy in ornithine carbamoyltransferase deficiency and other urea cycle disorders
-
Robinson, M.B., Batshaw, M.L., Ye, X., and Wilson, J.M. (1995b). Prospects for gene therapy in ornithine carbamoyltransferase deficiency and other urea cycle disorders. MRDS Res. Rev. 1:62-70.
-
(1995)
MRDS Res. Rev.
, vol.1
, pp. 62-70
-
-
Robinson, M.B.1
Batshaw, M.L.2
Ye, X.3
Wilson, J.M.4
-
51
-
-
0026610373
-
1A receptors are altered in the congenitally hyperammonemic sparse fur mouse
-
1A receptors are altered in the congenitally hyperammonemic sparse fur mouse. J. Neurochem. 58: 1016-10222.
-
(1992)
J. Neurochem.
, vol.58
, pp. 1016-10222
-
-
Robinson, M.B.1
Anegawa, N.J.2
Gorry, E.3
-
52
-
-
0026660831
-
Quinolinate in brain and cerebrospinal fluid in rat models of congenital hyperammonemia
-
Robinson, M.B., Heyes, M.P., Anegawa, N.J. et al. (1992b). Quinolinate in brain and cerebrospinal fluid in rat models of congenital hyperammonemia. Pediatr. Res. 32:483-488.
-
(1992)
Pediatr. Res.
, vol.32
, pp. 483-488
-
-
Robinson, M.B.1
Heyes, M.P.2
Anegawa, N.J.3
-
53
-
-
0020702374
-
Quinolinic acid: An endogenous metabolite that produces axon-sparing lesions in rat brain
-
Schwarcz, R., Whetsell, W.O., Jr., and Mangano, R.M. (1983). Quinolinic acid: an endogenous metabolite that produces axon-sparing lesions in rat brain. Science 219:316-318.
-
(1983)
Science
, vol.219
, pp. 316-318
-
-
Schwarcz, R.1
Whetsell Jr., W.O.2
Mangano, R.M.3
-
55
-
-
0021278998
-
A female case of ornithine transcarbamylase deficiency with marked computed tomographic abnormalities of the brain
-
Takayanagi, M., Ohtake, A., Ogura, N., Nakajima, H., and Hoshino, M. (1984). A female case of ornithine transcarbamylase deficiency with marked computed tomographic abnormalities of the brain. Brain Dev. 6:58.
-
(1984)
Brain Dev.
, vol.6
, pp. 58
-
-
Takayanagi, M.1
Ohtake, A.2
Ogura, N.3
Nakajima, H.4
Hoshino, M.5
-
56
-
-
0026597705
-
Orthotopic liver transplantation for urea cycle enzyme deficiency
-
Todo, S., Starzl, T.E., Tzakis, A., Benkov, K.J., Kalousek, F., Saheki, T., Tanikawa, K., and Fenton, W.A. (1992). Orthotopic liver transplantation for urea cycle enzyme deficiency. Hepatology 15:419-422.
-
(1992)
Hepatology
, vol.15
, pp. 419-422
-
-
Todo, S.1
Starzl, T.E.2
Tzakis, A.3
Benkov, K.J.4
Kalousek, F.5
Saheki, T.6
Tanikawa, K.7
Fenton, W.A.8
-
57
-
-
0022006126
-
Regulation of acetylcholine synthesis in the brain
-
Tucek, S. (1985). Regulation of acetylcholine synthesis in the brain. J. Neurochem. 44:11-24.
-
(1985)
J. Neurochem.
, vol.44
, pp. 11-24
-
-
Tucek, S.1
-
58
-
-
0030039992
-
Relative frequency of mutation causing ornithine transcarbamylase deficiency in 78 families
-
Tuchman, M., Plante, R.J., Garcia-Perez, M.A. and Rubio, V. (1996). Relative frequency of mutation causing ornithine transcarbamylase deficiency in 78 families. Human Genetics 97:274-276.
-
(1996)
Human Genetics
, vol.97
, pp. 274-276
-
-
Tuchman, M.1
Plante, R.J.2
Garcia-Perez, M.A.3
Rubio, V.4
-
59
-
-
0023663546
-
The molecular basis of the sparse fur mutation
-
Veres, G. Gibbs, R.A., Scherer, S.E. and Caskey, C.T. (1987). The molecular basis of the sparse fur mutation. Science 237:415-417.
-
(1987)
Science
, vol.237
, pp. 415-417
-
-
Veres, G.1
Gibbs, R.A.2
Scherer, S.E.3
Caskey, C.T.4
-
60
-
-
0030042794
-
Prolonged metabolic correction in adult ornithine transcarbamylase-deficient mice with adenoviral vectors
-
Ye, X., Robinson, M.B., Batshaw, M.L., Furth, E.E., Smith, I. and Wilson, J.M. (1996). Prolonged metabolic correction in adult ornithine transcarbamylase-deficient mice with adenoviral vectors. J. Biol. Chem. 271:3639-3646.
-
(1996)
J. Biol. Chem.
, vol.271
, pp. 3639-3646
-
-
Ye, X.1
Robinson, M.B.2
Batshaw, M.L.3
Furth, E.E.4
Smith, I.5
Wilson, J.M.6
|