Prolonged metabolic correction in adult ornithine transcarbamylase-deficient mice with adenoviral vectors.
about
AAV2/8-mediated correction of OTC deficiency is robust in adult but not neonatal Spf(ash) mice.Strategy of liver-directed gene therapy: present status and future prospects.Induction and prevention of severe hyperammonemia in the spfash mouse model of ornithine transcarbamylase deficiency using shRNA and rAAV-mediated gene delivery.Long-term regulated expression of growth hormone in mice after intramuscular gene transferPreclinical evaluation of a clinical candidate AAV8 vector for ornithine transcarbamylase (OTC) deficiency reveals functional enzyme from each persisting vector genome.Klf15 orchestrates circadian nitrogen homeostasis.Gene therapy of liver diseases.Role of viral antigens in destructive cellular immune responses to adenovirus vector-transduced cells in mouse lungs.Biology of adenovirus vectors with E1 and E4 deletions for liver-directed gene therapy.Sustained correction of OTC deficiency in spf( ash) mice using optimized self-complementary AAV2/8 vectors.Efficient mitochondrial import of newly synthesized ornithine transcarbamylase (OTC) and correction of secondary metabolic alterations in spf(ash) mice following gene therapy of OTC deficiency.Hepatocyte gene therapy in a large animal: a neonatal bovine model of citrullinemiaContrasting features of urea cycle disorders in human patients and knockout mouse models.Gene therapy for inborn errors of liver metabolism: progress towards clinical applicationsLysine 88 acetylation negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.Adenovirus-mediated gene delivery rescues a neonatal lethal murine model of mut(0) methylmalonic acidemia.Gene delivery to the rat liver using cationic lipid emulsion/DNA complex: comparison between intra-arterial, intraportal and intravenous administration.Liver-directed gene transfer and prolonged expression of three major human ApoE isoforms in ApoE-deficient mice.Phenotypic correction of ornithine transcarbamylase deficiency using low dose helper-dependent adenoviral vectors.Defective ureagenesis in mice carrying a liver-specific disruption of hepatocyte nuclear factor 4alpha (HNF4alpha ). HNF4alpha regulates ornithine transcarbamylase in vivo.Modeling correction of severe urea cycle defects in the growing murine liver using a hybrid recombinant adeno-associated virus/piggyBac transposase gene delivery system.Adeno-associated virus as a vector for liver-directed gene therapy.Activation of transgene expression by early region 4 is responsible for a high level of persistent transgene expression from adenovirus vectors in vivo.AAV gene therapy corrects OTC deficiency and prevents liver fibrosis in aged OTC-knock out heterozygous mice.Elimination of cholesterol ester from macrophage foam cells by adenovirus-mediated gene transfer of hormone-sensitive lipase.AAV-encoded OTC activity persisting to adulthood following delivery to newborn spf(ash) mice is insufficient to prevent shRNA-induced hyperammonaemia.In vivo assessment of mutations in OTC for dominant-negative effects following rAAV2/8-mediated gene delivery to the mouse liver.Humoral immune response to recombinant adenovirus and adeno-associated virus after in utero administration of viral vectors in mice.Effects of nonlipolytic ligand function of endothelial lipase on high density lipoprotein metabolism in vivo.Tissue-specific consequences of the anti-adenoviral immune response: implications for cardiac transplants.Correction of ureagenesis after gene transfer in an animal model and after liver transplantation in humans with ornithine transcarbamylase deficiency.Cotransfection of heme oxygenase-1 prevents the acute inflammation elicited by a second adenovirus.Transient depletion of CD4 lymphocyte improves efficacy of repeated administration of recombinant adenovirus in the ornithine transcarbamylase deficient sparse fur mouse.Gene therapy for metabolic disorders: an overview with a focus on urea cycle disorders.Adenovirus-mediated in vivo gene transfer rapidly protects ornithine transcarbamylase-deficient mice from an ammonium challenge.
P2860
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P2860
Prolonged metabolic correction in adult ornithine transcarbamylase-deficient mice with adenoviral vectors.
description
1996 nî lūn-bûn
@nan
1996年の論文
@ja
1996年学术文章
@wuu
1996年学术文章
@zh
1996年学术文章
@zh-cn
1996年学术文章
@zh-hans
1996年学术文章
@zh-my
1996年学术文章
@zh-sg
1996年學術文章
@yue
1996年學術文章
@zh-hant
name
Prolonged metabolic correction ...... mice with adenoviral vectors.
@en
Prolonged metabolic correction ...... mice with adenoviral vectors.
@nl
type
label
Prolonged metabolic correction ...... mice with adenoviral vectors.
@en
Prolonged metabolic correction ...... mice with adenoviral vectors.
@nl
prefLabel
Prolonged metabolic correction ...... mice with adenoviral vectors.
@en
Prolonged metabolic correction ...... mice with adenoviral vectors.
@nl
P2093
P2860
P356
P1476
Prolonged metabolic correction ...... mice with adenoviral vectors.
@en
P2093
P2860
P304
P356
10.1074/JBC.271.7.3639
P407
P577
1996-02-01T00:00:00Z