Warning: Undefined array key "mm" in /www/wwwroot/www.ai-bt.com/si.php on line 10 Deprecated: trim(): Passing null to parameter #1 ($string) of type string is deprecated in /www/wwwroot/www.ai-bt.com/si.php on line 10 Ornithine transcarbamylase deficiency in mutant mice I. Studies on the characterization of enzyme defect and suitability as animal model of human disease.

× No keyword cloud information.

13 in total

1. Gene therapy for metabolic disorders: an overview with a focus on urea cycle disorders.

Authors: Ian E Alexander; Cindy Kok; Allison P Dane; Sharon C Cunningham
Journal: J Inherit Metab Dis Date: 2012-03-09 Impact factor: 4.982

2. Differential inhibition by hyperammonemia of the electron transport chain enzymes in synaptosomes and non-synaptic mitochondria in ornithine transcarbamylase-deficient spf-mice: restoration by acetyl-L-carnitine.

Authors: K Qureshi; K V Rao; I A Qureshi
Journal: Neurochem Res Date: 1998-06 Impact factor: 3.996

3. Activity of orotate metabolizing enzyme complex and various urea-cycle enzymes in mutant mice with ornithine transcarbamylase deficiency.

Authors: I A Qureshi; J Letarte; R Ouellet
Journal: Experientia Date: 1982-03-15

4. The spfash mouse: a missense mutation in the ornithine transcarbamylase gene also causes aberrant mRNA splicing.

Authors: P E Hodges; L E Rosenberg
Journal: Proc Natl Acad Sci U S A Date: 1989-06 Impact factor: 11.205

5. Structure of the rat ornithine carbamoyltransferase gene, a large, X chromosome-linked gene with an atypical promoter.

Authors: M Takiguchi; T Murakami; S Miura; M Mori
Journal: Proc Natl Acad Sci U S A Date: 1987-09 Impact factor: 11.205

6. Developing adenoviral-mediated in vivo gene therapy for ornithine transcarbamylase deficiency.

Authors: S E Raper; J M Wilson; M Yudkoff; M B Robinson; X Ye; M L Batshaw
Journal: J Inherit Metab Dis Date: 1998 Impact factor: 4.982

7. Loss of [3H]MK801 binding sites in brain in congenital ornithine transcarbamylase deficiency.

Authors: L Ratnakumari; I A Qureshi; R F Butterworth
Journal: Metab Brain Dis Date: 1995-09 Impact factor: 3.584

8. AAV gene therapy corrects OTC deficiency and prevents liver fibrosis in aged OTC-knock out heterozygous mice.

Authors: Lili Wang; Peter Bell; Hiroki Morizono; Zhenning He; Elena Pumbo; Hongwei Yu; John White; Mark L Batshaw; James M Wilson
Journal: Mol Genet Metab Date: 2017-03-02 Impact factor: 4.797

9. Regional amino acid neurotransmitter changes in brains of spf/Y mice with congenital ornithine transcarbamylase deficiency.

Authors: L Ratnakumari; I A Qureshi; R F Butterworth
Journal: Metab Brain Dis Date: 1994-03 Impact factor: 3.584

10. Efficient adenoviral-mediated ornithine transcarbamylase expression in deficient mouse and human hepatocytes.

Authors: M A Morsy; E L Alford; A Bett; F L Graham; C T Caskey
Journal: J Clin Invest Date: 1993-09 Impact factor: 14.808