Contrasting features of urea cycle disorders in human patients and knockout mouse models.
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Suggested guidelines for the diagnosis and management of urea cycle disordersA loose domain swapping organization confers a remarkable stability to the dimeric structure of the arginine binding protein from Thermotoga maritimaStrategies to rescue the consequences of inducible arginase-1 deficiency in miceAmmonia toxicity to the brain.Two hypomorphic alleles of mouse Ass1 as a new animal model of citrullinemia type I and other hyperammonemic syndromes.Arginase in retinopathy.Induction and prevention of severe hyperammonemia in the spfash mouse model of ornithine transcarbamylase deficiency using shRNA and rAAV-mediated gene delivery.Remodeling of hepatic metabolism and hyperaminoacidemia in mice deficient in proglucagon-derived peptides.A novel biochemically salvageable animal model of hyperammonemia devoid of N-acetylglutamate synthaseAdeno-associated virus-mediated rescue of neonatal lethality in argininosuccinate synthetase-deficient miceRestoring Ureagenesis in Hepatocytes by CRISPR/Cas9-mediated Genomic Addition to Arginase-deficient Induced Pluripotent Stem Cells.Arginase: an old enzyme with new tricks.Generation of carbamoyl phosphate synthetase 1 reporter cell lines for the assessment of ammonia metabolism.Modeling correction of severe urea cycle defects in the growing murine liver using a hybrid recombinant adeno-associated virus/piggyBac transposase gene delivery system.Arginase Inhibitors: A Rational Approach Over One Century.Late-onset ornithine transcarbamylase deficiency: An under recognized cause of metabolic encephalopathy.Critical role for arginase 2 in obesity-associated pancreatic cancer.Somatic genome editing with CRISPR/Cas9 generates and corrects a metabolic disease.Metabolic profiling of presymptomatic Huntington's disease sheep reveals novel biomarkers.Gene therapy for metabolic disorders: an overview with a focus on urea cycle disorders.Urea cycle disorder--argininosuccinic lyase deficiency.
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P2860
Contrasting features of urea cycle disorders in human patients and knockout mouse models.
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
2007年论文
@zh
2007年论文
@zh-cn
name
Contrasting features of urea cycle disorders in human patients and knockout mouse models.
@en
type
label
Contrasting features of urea cycle disorders in human patients and knockout mouse models.
@en
prefLabel
Contrasting features of urea cycle disorders in human patients and knockout mouse models.
@en
P2093
P2860
P1476
Contrasting features of urea cycle disorders in human patients and knockout mouse models.
@en
P2093
Joshua L Deignan
Stephen D Cederbaum
Wayne W Grody
P2860
P356
10.1016/J.YMGME.2007.08.123
P577
2007-10-22T00:00:00Z