Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion
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Suggested guidelines for the diagnosis and management of urea cycle disordersClinical and experimental applications of sodium phenylbutyrateEvidence of a vicious cycle in glutamine synthesis and breakdown in pathogenesis of hepatic encephalopathy-therapeutic perspectives.Switch from Sodium Phenylbutyrate to Glycerol Phenylbutyrate Improved Metabolic Stability in an Adolescent with Ornithine Transcarbamylase DeficiencyAmmonia control in children ages 2 months through 5 years with urea cycle disorders: comparison of sodium phenylbutyrate and glycerol phenylbutyrate.ER retention is imposed by COPII protein sorting and attenuated by 4-phenylbutyrate.Phase 2 comparison of a novel ammonia scavenging agent with sodium phenylbutyrate in patients with urea cycle disorders: safety, pharmacokinetics and ammonia control.Modifying histones to tame cancer: clinical development of sodium phenylbutyrate and other histone deacetylase inhibitors.Branched-chain amino acid metabolism: from rare Mendelian diseases to more common disorders.Sodium phenylbutyrate decreases plasma branched-chain amino acids in patients with urea cycle disorders.A zebrafish model of hyperammonemia.Glutamine and hyperammonemic crises in patients with urea cycle disorders.Phenyl acyl acids attenuate the unfolded protein response in tunicamycin-treated neuroblastoma cellsPhenylbutyrate improves nitrogen disposal via an alternative pathway without eliciting an increase in protein breakdown and catabolism in control and ornithine transcarbamylase-deficient patients.Interpreting clinical assays for histone deacetylase inhibitors.Glycerol phenylbutyrate treatment in children with urea cycle disorders: pooled analysis of short and long-term ammonia control and outcomes.Emerging drug treatments for cystic fibrosis.Connectivity mapping using a combined gene signature from multiple colorectal cancer datasets identified candidate drugs including existing chemotherapiesTopical ocular sodium 4-phenylbutyrate rescues glaucoma in a myocilin mouse model of primary open-angle glaucoma.Acute depletion of plasma glutamine increases leucine oxidation in prednisone-treated humans.Renal systems biology of patients with systemic inflammatory response syndromeSodium 4-phenylbutyrate prevents murine dietary steatohepatitis caused by trans-fatty acid plus fructose.Ammonia control and neurocognitive outcome among urea cycle disorder patients treated with glycerol phenylbutyrate.Targeted therapy for cystic fibrosis: cystic fibrosis transmembrane conductance regulator mutation-specific pharmacologic strategies.Urinary phenylacetylglutamine as dosing biomarker for patients with urea cycle disorders.New insights in nutritional management and amino acid supplementation in urea cycle disordersAmmonia control in children with urea cycle disorders (UCDs); phase 2 comparison of sodium phenylbutyrate and glycerol phenylbutyrate.Pharmacology and safety of glycerol phenylbutyrate in healthy adults and adults with cirrhosis.Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency.Epigenetic mechanisms in cerebral ischemia.Urinary Metabolic Phenotyping Reveals Differences in the Metabolic Status of Healthy and Inflammatory Bowel Disease (IBD) Children in Relation to Growth and Disease Activity.In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.Hyperammonemia crisis following parturition in a female patient with ornithine transcarbamylase deficiency.Impact of Histone Deacetylase Inhibitors on microRNA Expression and Cancer Therapy: A Review.An update on the use of benzoate, phenylacetate and phenylbutyrate ammonia scavengers for interrogating and modifying liver nitrogen metabolism and its implications in urea cycle disorders and liver disease.Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin.Clinical manifestations and growth of patients with urea cycle disorders in Japan.Profile of sodium phenylbutyrate granules for the treatment of urea-cycle disorders: patient perspectives.Identification of enzymes involved in oxidation of phenylbutyrate.Increased levels of urinary phenylacetylglycine associated with mitochondrial toxicity in a model of drug-induced phospholipidosis.
P2860
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P2860
Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion
description
1991 nî lūn-bûn
@nan
1991 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
1991 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
1991年の論文
@ja
1991年論文
@yue
1991年論文
@zh-hant
1991年論文
@zh-hk
1991年論文
@zh-mo
1991年論文
@zh-tw
1991年论文
@wuu
name
Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion
@ast
Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion
@en
type
label
Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion
@ast
Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion
@en
prefLabel
Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion
@ast
Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion
@en
P3181
P1433
P1476
Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion
@en
P2093
Saul W Brusilow
P304
P3181
P356
10.1203/00006450-199102000-00009
P407
P577
1991-02-01T00:00:00Z
P5875
P6179
1011922398