-
1
-
-
34547387770
-
Congenital hyperammonemia (Model No. 235)
-
C. C. Capen, D. B Hackle, T. C Jones, G. Migaki ed., Fase 11. Washington, D.C: Registry of Comparative Pathology
-
I.A. Qureshi, J. Letarle, and S.R. Qureshi, Congenital hyperammonemia (Model No. 235), in: Handbook of Animal Models of Human Disease, C. C. Capen, D. B Hackle, T. C Jones, G. Migaki ed., Fase 11. Washington, D.C: Registry of Comparative Pathology, pp 2-4 (1981).
-
(1981)
Handbook of Animal Models of Human Disease
, pp. 2-4
-
-
Qureshi, I.A.1
Letarle, J.2
Qureshi, S.R.3
-
2
-
-
0041960733
-
Animal Models of congenital hyperammonemia
-
J.T.Coyle, ed., Alan. R. Liss, Inc, New York
-
M.L. Batshaw, S.L. Hyman, C. Bachmann, I.A. Qureshi and J.T. Coyle, Animal Models of congenital hyperammonemia, in: Animal Models of Dementia, J.T.Coyle, ed., Alan. R. Liss, Inc, New York, pp 163-198 (1987).
-
(1987)
Animal Models of Dementia
, pp. 163-198
-
-
Batshaw, M.L.1
Hyman, S.L.2
Bachmann, C.3
Qureshi, I.A.4
Coyle, J.T.5
-
3
-
-
84913987925
-
Congenital hyperammonemia (Model No. 235) Supplemetal Update
-
C. C. Capen, D. B Hackle, T. C Jones, G. Migaki, ed., Fase 11. Washington, D.C: Registry of Comparative Pathology
-
I.A. Qureshi, Congenital hyperammonemia (Model No. 235) Supplemetal Update, in: Handbook of Animal Models of Human Disease, C. C. Capen, D. B Hackle, T. C Jones, G. Migaki, ed., Fase 11. Washington, D.C: Registry of Comparative Pathology, pp 1-2 (1989).
-
(1989)
Handbook of Animal Models of Human Disease
, pp. 1-2
-
-
Qureshi, I.A.1
-
4
-
-
0042962457
-
Animal models of hereditary hyperammonemias
-
A. Boulton., G. Baker and R. Butterworth, ed., The Humana Press Inc, New York
-
I.A. Qureshi, Animal models of hereditary hyperammonemias, in: Neuromethods, Animal Models of Neurological Disease, II, A. Boulton., G. Baker and R. Butterworth, ed., The Humana Press Inc, New York pp 329-356 (1992).
-
(1992)
Neuromethods, Animal Models of Neurological Disease, II
, pp. 329-356
-
-
Qureshi, I.A.1
-
5
-
-
0042461295
-
Abnormal ornithine carbamoyl-transferase in mice having the sparse-fur mutation
-
U.S.A.
-
R. Demars, S.L. LeVan, B.L. Trend and L.B. Russel, Abnormal ornithine carbamoyl-transferase in mice having the sparse-fur mutation. Proc. Natl. Acad. Sci, U.S.A. 73:1693-1698 (1976).
-
(1976)
Proc. Natl. Acad. Sci
, vol.73
, pp. 1693-1698
-
-
Demars, R.1
Levan, S.L.2
Trend, B.L.3
Russel, L.B.4
-
6
-
-
0042962459
-
Spontaneous animal models of ornithine transcarbamylase deficiency: Studies on serum and urinary nitrogen metabolites
-
A. Lowenthal, A. Mori and B. Marecau, ed., Plenum Press, New York
-
I. A. Qureshi, J. Letarte and R. Quellet, Spontaneous animal models of ornithine transcarbamylase deficiency: Studies on serum and urinary nitrogen metabolites, in: Urea Cycle Diseases, A. Lowenthal, A. Mori and B. Marecau, ed., Plenum Press, New York, pp 173-183 (1983).
-
(1983)
Urea Cycle Diseases
, pp. 173-183
-
-
Qureshi, I.A.1
Letarte, J.2
Quellet, R.3
-
7
-
-
0029561659
-
The sparse-fur mouse as a model of gene therapy in ornithine carbamoyltransferase deficiency
-
M.L. Batshaw, M. Yudkoff, B.A. McLaughlin, E. Gorry, NJ. Anegawa, I.A.S. Smith and M.B. Robinson, The sparse-fur mouse as a model of gene therapy in ornithine carbamoyltransferase deficiency, Gene Therapy. 2:743-749 (1995).
-
(1995)
Gene Therapy
, vol.2
, pp. 743-749
-
-
Batshaw, M.L.1
Yudkoff, M.2
McLaughlin, B.A.3
Gorry, E.4
Anegawa, N.J.5
Smith, I.A.S.6
Robinson, M.B.7
-
8
-
-
0029042918
-
Efficient retroviral-mediated gene transfer into primary cultures of murine and human hepatocytes: Expression of LDL receptor
-
J.C. Pages, M. Andreoletti, M. Bennoun, C. Vons, J. Elcheroth, P. Lehn, D. Houssin, J. Chapman, P. Briand, R. Benarous, D. Franco and A. Weber, Efficient retroviral-mediated gene transfer into primary cultures of murine and human hepatocytes: Expression of LDL receptor, Human Gene Therapy 6:21-30 (1995).
-
(1995)
Human Gene Therapy
, vol.6
, pp. 21-30
-
-
Pages, J.C.1
Andreoletti, M.2
Bennoun, M.3
Vons, C.4
Elcheroth, J.5
Lehn, P.6
Houssin, D.7
Chapman, J.8
Briand, P.9
Benarous, R.10
Franco, D.11
Weber, A.12
-
9
-
-
0029039893
-
Hepatocyte transplantation and gene therapy
-
S.E. Raper, Hepatocyte transplantation and gene therapy, Clin Transplantation. 9:249-254 (1995).
-
(1995)
Clin Transplantation.
, vol.9
, pp. 249-254
-
-
Raper, S.E.1
-
10
-
-
0028567119
-
Ornithine transcarbamylase deficiency: A model for gene therapy
-
V. Felipo and S. Grisolia, ed., Plenum Press, New York
-
M.A. Morsy and C.T. Caskey, Ornithine transcarbamylase deficiency: A model for gene therapy, in: Hepatic Encephalopathy, Hyperammonemia, and Ammonia Toxicity, V. Felipo and S. Grisolia, ed., Plenum Press, New York, pp 145-154 (1994).
-
(1994)
Hepatic Encephalopathy, Hyperammonemia, and Ammonia Toxicity
, pp. 145-154
-
-
Morsy, M.A.1
Caskey, C.T.2
-
11
-
-
0030040201
-
Patient selection may affect gene therapy success
-
M.A. Morsy, J.Z. Zhao, T.T. Ngo, A.W. Warman, W.E. O'Brien and F.L. Graham, Patient selection may affect gene therapy success, J. Clin. Invest. 97:826-831 (1996).
-
(1996)
J. Clin. Invest.
, vol.97
, pp. 826-831
-
-
Morsy, M.A.1
Zhao, J.Z.2
Ngo, T.T.3
Warman, A.W.4
O'Brien, W.E.5
Graham, F.L.6
-
12
-
-
0030042794
-
Prolonged metabolic correction in adult ornithine transcarbamylase-deficient mice with adenoviral vectors
-
X. Ye, M.B. Robinson, M.L. Batshaw, E.E. Furth, I. Smith and J.M. Wilson, Prolonged metabolic correction in adult ornithine transcarbamylase-deficient mice with adenoviral vectors, J. Biol. Chem. 271:3639-3646 (1996).
-
(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
-
13
-
-
0018384552
-
Ornithine transcarbamylase deficiency in mutant mice I. Studies on the characterization of enzyme defect and suitability as animal model of human disease
-
I.A. Qureshi, J. Letarte and R. Quellet, Ornithine transcarbamylase deficiency in mutant mice I. Studies on the characterization of enzyme defect and suitability as animal model of human disease, Pediat. Res. 13:807-811 (1979).
-
(1979)
Pediat. Res.
, vol.13
, pp. 807-811
-
-
Qureshi, I.A.1
Letarte, J.2
Quellet, R.3
-
14
-
-
0023663546
-
The molecular basis of sparse-fur mouse mutation
-
G. Veres, R.A. Gibbs, S.E. Scherer and C.T. Caskey, The molecular basis of sparse-fur mouse mutation, Science. 237:415-417(1987).
-
(1987)
Science.
, vol.237
, pp. 415-417
-
-
Veres, G.1
Gibbs, R.A.2
Scherer, S.E.3
Caskey, C.T.4
-
16
-
-
0021098084
-
Cell-free synthesis and transport of precursors of mutant ornithine carbamoyltransferases into mitochondria
-
P. Briand, S. Mirira, M. Mori, L. Cathelineau, P. Kamoun and M. Talibana, Cell-free synthesis and transport of precursors of mutant ornithine carbamoyltransferases into mitochondria, Biochem. Biophys. Acta, 760:389-397(1983).
-
(1983)
Biochem. Biophys. Acta
, vol.760
, pp. 389-397
-
-
Briand, P.1
Mirira, S.2
Mori, M.3
Cathelineau, L.4
Kamoun, P.5
Talibana, M.6
-
17
-
-
0023653348
-
ash mice: Genes, mRNA and mRNA precursors
-
ash mice: Genes, mRNA and mRNA precursors, Biochem. Biophys. Res. Commun. 146:1064-1070 (1987).
-
(1987)
Biochem. Biophys. Res. Commun.
, vol.146
, pp. 1064-1070
-
-
Ohtake, A.1
Takayanagi, M.2
Yamamoto, S.3
Nakajima, H.4
Mori, M.5
-
18
-
-
0023759132
-
Compared expression of ornithine transcarbamylase and carbamyl phosphate synthetase in liver and small intestine of normal and mutant mice
-
N. Dubois, C. Cavard, J.F. Chasse, P. Kamoun and P. Briand, Compared expression of ornithine transcarbamylase and carbamyl phosphate synthetase in liver and small intestine of normal and mutant mice, Biochim. Biophys. Acta. 950:321-328 (1988).
-
(1988)
Biochim. Biophys. Acta.
, vol.950
, pp. 321-328
-
-
Dubois, N.1
Cavard, C.2
Chasse, J.F.3
Kamoun, P.4
Briand, P.5
-
19
-
-
34547388045
-
Enhanced expression of hepatic mitochondrial urea cycle enzymes and cytochrome C oxidase with chronic acetyl-L-carnitine treatment in spf mice with ornithine transcarbamylase deficiency
-
submitted
-
Y.R. Mawal, K.V. RamaRao and I.A. Qureshi, Enhanced expression of hepatic mitochondrial urea cycle enzymes and cytochrome C oxidase with chronic acetyl-L-carnitine treatment in spf mice with ornithine transcarbamylase deficiency, J. Biol. Chem. (submitted) (1996).
-
(1996)
J. Biol. Chem.
-
-
Mawal, Y.R.1
Ramarao, K.V.2
Qureshi, I.A.3
-
20
-
-
0000467069
-
Hyperammonemia. a new instance of an inborn enzymatic defect of the biosynthesis of urea
-
A. Russel, B. Levin, V.G. Oberholzer and L. Sinclair, Hyperammonemia. A new instance of an inborn enzymatic defect of the biosynthesis of urea, Lancet. 2:699-700 (1962).
-
(1962)
Lancet.
, vol.2
, pp. 699-700
-
-
Russel, A.1
Levin, B.2
Oberholzer, V.G.3
Sinclair, L.4
-
21
-
-
0015913712
-
Evidence for X-linked dominant inheritance of ornithine transcarbamylase deficiency
-
E.M. Short, HO. Conn, P.J. Snodgrass, A.G.M. Campbell and L.E. Rosenberg, Evidence for X-linked dominant inheritance of ornithine transcarbamylase deficiency, N. Engl. J. Med. 288:7-12 (1973).
-
(1973)
N. Engl. J. Med.
, vol.288
, pp. 7-12
-
-
Short, E.M.1
Conn, H.O.2
Snodgrass, P.J.3
Campbell, A.G.M.4
Rosenberg, L.E.5
-
22
-
-
0020316641
-
Omithine transcarbamylase deficiencies in human males: Kinetic and immunochemical classification
-
P. Briand, B. Francois, D. Rabier and L. Cathelineau, Omithine transcarbamylase deficiencies in human males: Kinetic and immunochemical classification, Biochem. Biophys. Acta. 704:100-106 (1982).
-
(1982)
Biochem. Biophys. Acta.
, vol.704
, pp. 100-106
-
-
Briand, P.1
Francois, B.2
Rabier, D.3
Cathelineau, L.4
-
23
-
-
0021831960
-
Expression of ornithine tianscarbamylase deficiency in the small intestine and colon of sparse-fur mutant mice
-
I.A. Qureshi, J. Letarte and R. Quellet, Expression of ornithine tianscarbamylase deficiency in the small intestine and colon of sparse-fur mutant mice, J. Pediatr. Gastroenterol. Nutr. 4:118-124 (1985).
-
(1985)
J. Pediatr. Gastroenterol. Nutr.
, vol.4
, pp. 118-124
-
-
Qureshi, I.A.1
Letarte, J.2
Quellet, R.3
-
24
-
-
34547387629
-
The sparse-fur mouse: An animal model for a human inborn error of metabolism of the urea cycle, in: Orphan Drugs and Orphan Diseases: Clinical Realities and Public Policy
-
New York
-
E.B. Spector and R.A. Mazzochi, The sparse-fur mouse: An animal model for a human inborn error of metabolism of the urea cycle, in: Orphan Drugs and Orphan Diseases: Clinical Realities and Public Policy, Alan R. Liss, Inc, New York, pp 86-96 (1983).
-
(1983)
Alan R. Liss, Inc
, pp. 86-96
-
-
Spector, E.B.1
Mazzochi, R.A.2
-
25
-
-
0020482841
-
Activity of orotate metabolizing enzyme complex and various urea cycle enzymes in mutant mice with ornithinc trans-carbamylase deficiency
-
I.A. Qureshi, J. Letarte and R. Ouellet, Activity of orotate metabolizing enzyme complex and various urea cycle enzymes in mutant mice with ornithinc trans-carbamylase deficiency, Experientia. 38:308-309 (1982).
-
(1982)
Experientia
, vol.38
, pp. 308-309
-
-
Qureshi, I.A.1
Letarte, J.2
Ouellet, R.3
-
26
-
-
0026765489
-
Abnormal hepatic nucleotide pools in sparse-fur (spl) mutant mice deficient in ornithine transcarbamylase
-
S. Vasudevan, I.A. Qureshi, L. Mores, P.M. Rao, S. Rajalakshmi and D.S.R. Sarma, Abnormal hepatic nucleotide pools in sparse-fur (spl) mutant mice deficient in ornithine transcarbamylase, Biochem Med. Metabol. Biol. 47:274-278(1992).
-
(1992)
Biochem Med. Metabol. Biol.
, vol.47
, pp. 274-278
-
-
Vasudevan, S.1
Qureshi, I.A.2
Mores, L.3
Rao, P.M.4
Rajalakshmi, S.5
Sarma, D.S.R.6
-
27
-
-
0029553689
-
Nucleotide pool imbalances in the livers of patients with urea cycle disorders associated with increased levels of orotic acid
-
L. Vasudevan, I.A. Qureshi, M. Lambert, P. Rao, S. Rajalakshmi and D.S.R. Sarma, Nucleotide pool imbalances in the livers of patients with urea cycle disorders associated with increased levels of orotic acid, Biochem. Mol. Biol. Int. 35:685-690 (1995).
-
(1995)
Biochem. Mol. Biol. Int.
, vol.35
, pp. 685-690
-
-
Vasudevan, L.1
Qureshi, I.A.2
Lambert, M.3
Rao, P.4
Rajalakshmi, S.5
Sarma, D.S.R.6
-
28
-
-
0022994785
-
Variablite de l'active enzymatique et de l'acidurie orotique chez les souris spf/+ heterozygotes deficientes en ornithine transcarbamylase
-
I.A. Qureshi, J. Letarte, S. Lebel and R. Ouellet, Variablite de l'active enzymatique et de l'acidurie orotique chez les souris spf/+ heterozygotes deficientes en ornithine transcarbamylase, Diabete. Metabolisme. 12:250-255 (1986).
-
(1986)
Diabete. Metabolisme.
, vol.12
, pp. 250-255
-
-
Qureshi, I.A.1
Letarte, J.2
Lebel, S.3
Ouellet, R.4
-
29
-
-
0029974447
-
Chronic sodium benzoate therapy in children with inborn errors of urea synthesis: Effect on carnitine metabolism and ammonia nitrogen removal
-
J.C. Feoli-Fonscca, M. Lambert, G. Mitchell, S.B. Melançon, L. Dallaire, D.S. Millington and I.A. Qureshi, Chronic sodium benzoate therapy in children with inborn errors of urea synthesis: Effect on carnitine metabolism and ammonia nitrogen removal, Biochem. Mol. Med. 57:31-36 (1996).
-
(1996)
Biochem. Mol. Med.
, vol.57
, pp. 31-36
-
-
Feoli-Fonscca, J.C.1
Lambert, M.2
Mitchell, G.3
Melançon, S.B.4
Dallaire, L.5
Millington, D.S.6
Qureshi, I.A.7
-
30
-
-
0027362897
-
Carnitine musculaire chez les souris hyperammoniémiques: Effect du traitement au benzoate de sodium
-
A. Michalak and I.A. Qureshi, Carnitine musculaire chez les souris hyperammoniémiques: effect du traitement au benzoate de sodium, Can. J. Physiol. Pharmacol. 71:439-446 (1990).
-
(1990)
Can. J. Physiol. Pharmacol.
, vol.71
, pp. 439-446
-
-
Michalak, A.1
Qureshi, I.A.2
-
31
-
-
0027792109
-
Profil des acylcamitines hepatiques et musculares chez les souris chroniquement hyperammonemiques apres un traitment aigu avec le benzoate de sodium: Etudes dose-response
-
A. Michalak and I.A. Qureshi, Profil des acylcamitines hepatiques et musculares chez les souris chroniquement hyperammonemiques apres un traitment aigu avec le benzoate de sodium: etudes dose-response, Ann Biol Clin. 50:879-885 (1993).
-
(1993)
Ann Biol Clin.
, vol.50
, pp. 879-885
-
-
Michalak, A.1
Qureshi, I.A.2
-
32
-
-
0023478104
-
Accumulation of large neutral amino acids in the brains of sparse-fur mice at hyperammonemic state
-
I. Inoue, T. Gushiken, K. Kobayashi and T. Saheki, Accumulation of large neutral amino acids in the brains of sparse-fur mice at hyperammonemic state, Biochem Med. Metabol. Biol. 38:378-386 (1987).
-
(1987)
Biochem Med. Metabol. Biol.
, vol.38
, pp. 378-386
-
-
Inoue, I.1
Gushiken, T.2
Kobayashi, K.3
Saheki, T.4
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