Clinical phenotype, biochemical profile, and treatment in 19 patients with arginase 1 deficiency

Biosynthesis of homoarginine (hArg) and asymmetric dimethylarginine (ADMA) from acutely and chronically administered free l-arginine in humans

(2015) Arslan Arinc Kayacelebi et al.   AMINO ACIDS

Hyperargininemia due to arginase I deficiency: the original patients and their natural history, and a review of the literature

(2015) A. Schlune et al.   AMINO ACIDS

Redox signalling and mitochondrial stress responses; lessons from inborn errors of metabolism

(2015) Rikke K. J. Olsen et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Treatment of arginase deficiency revisited: guanidinoacetate as a therapeutic target and biomarker for therapeutic monitoring

(2014) Wajdi Amayreh et al.   DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY

Quantification of Arginine and its Methylated Derivatives in Healthy Children by Liquid Chromatography-Tandem Mass Spectrometry

(2014) Fernando Andrade et al.   JOURNAL OF CHROMATOGRAPHIC SCIENCE

Arginase-1 Deficiency Regulates Arginine Concentrations and NOS2-Mediated NO Production during Endotoxemia

(2014) Karolina A. P. Wijnands et al.   PLoS One

l-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders

(2013) Graziela S. Ribas et al.   GENE

Glutathione metabolism in cobalamin deficiency type C (cblC)

(2013) Anna Pastore et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Cross-sectional observational study of 208 patients with non-classical urea cycle disorders

(2013) Corinne M. Rüegger et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Creatine and guanidinoacetate reference values in a French population

(2013) Marie Joncquel-Chevalier Curt et al.   MOLECULAR GENETICS AND METABOLISM

Dietary management of urea cycle disorders: UK practice

(2012) S. Adam et al.   JOURNAL OF HUMAN NUTRITION AND DIETETICS

Clinical Features and Neurologic Progression of Hyperargininemia

(2012) Daniel R. Carvalho et al.   PEDIATRIC NEUROLOGY

Protective effect of antioxidants on blood oxidative stress caused by arginine

(2011) Daniela Delwing de Lima et al.   FUNDAMENTAL & CLINICAL PHARMACOLOGY

Reference Intervals for Plasma L-Arginine and the L-Arginine:Asymmetric Dimethylarginine Ratio in the Framingham Offspring Cohort

(2011) Nicole Lüneburg et al.   JOURNAL OF NUTRITION

Review article: Asymmetric dimethylarginine as a mediator of vascular dysfunction and a marker of cardiovascular disease and mortality: an intriguing interaction with diabetes mellitus

(2010) Maike Anderssohn et al.   Diabetes & Vascular Disease Research

Creatine prevents the inhibition of energy metabolism and lipid peroxidation in rats subjected to GAA administration

(2010) Janaína Kolling et al.   METABOLIC BRAIN DISEASE

Guanidino compound levels in blood, cerebrospinal fluid, and post-mortem brain material of patients with argininemia

(2010) Joshua L. Deignan et al.   MOLECULAR GENETICS AND METABOLISM

Evaluation of endogenous nitric oxide synthesis in congenital urea cycle enzyme defects

(2009) Hironori Nagasaka et al.   METABOLISM-CLINICAL AND EXPERIMENTAL

Profiling of oxidative stress in patients with inborn errors of metabolism

(2009) Peter J. Mc Guire et al.   MOLECULAR GENETICS AND METABOLISM

Amino acids in CSF and plasma in hyperammonaemic coma due to arginase1 deficiency

(2008) S. Scholl-Bürgi et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Guanidinoacetate Decreases Antioxidant Defenses and Total Protein Sulfhydryl Content in Striatum of Rats

(2008) Alexandra I. Zugno et al.   NEUROCHEMICAL RESEARCH

Protective effect of nitric oxide synthase inhibition or antioxidants on brain oxidative damage caused by intracerebroventricular arginine administration

(2007) Débora Delwing et al.   BRAIN RESEARCH

Increased plasma and tissue guanidino compounds in a mouse model of hyperargininemia

(2007) Joshua L. Deignan et al.   MOLECULAR GENETICS AND METABOLISM