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George A. Díaz

Icahn School of Medicine at Mount Sinai · US
Area of research
Clinical Biochemistry · Genetics
Research interest
Research interests include Metabolism and Genetic Disorders, Genomics and Rare Diseases, BRCA gene mutations in cancer, and Folate and B Vitamins Research.
h-index
44
citations
7,091
works
182
NIH funding
primary concept
email

Recent publications

Efficacy and safety of pegzilarginase in arginase 1 deficiency (PEACE): a phase 3, randomized, double-blind, placebo-controlled, multi-centre trial
EClinicalMedicine 2024cited by 29position: middledoi
Long-term safety and clinical outcomes of olipudase alfa enzyme replacement therapy in pediatric patients with acid sphingomyelinase deficiency: two-year results.
2022cited by 34position: contributordoi
The role and control of arginine levels in arginase 1 deficiency
Journal of Inherited Metabolic Disease 2022cited by 27position: firstdoi
Health insurance literacy and health services access barriers in Niemann-Pick disease: the patient and caregiver voice.
2022cited by 3position: contributordoi
Health care resource utilization in the management of patients with Arginase 1 Deficiency in the US: a retrospective, observational, claims database study.
2022cited by 3position: contributordoi
One-year results of a clinical trial of olipudase alfa enzyme replacement therapy in pediatric patients with acid sphingomyelinase deficiency.
2021cited by 54position: contributordoi
Clinical effect and safety profile of pegzilarginase in patients with arginase 1 deficiency.
2021cited by 28position: contributordoi
Long-term safety and efficacy of glycerol phenylbutyrate for the management of urea cycle disorder patients
Molecular Genetics and Metabolism 2019cited by 19position: firstdoi
Biochemical signatures mimicking multiple carboxylase deficiency in children with mutations in MT-ATP6
Mitochondrion 2018cited by 27position: middledoi
Institutional Profile: Translational Pharmacogenomics at the Icahn School of Medicine at Mount Sinai
Pharmacogenomics 2017cited by 21position: middledoi
Analysis of 589,306 genomes identifies individuals resilient to severe Mendelian childhood diseases
Nature Biotechnology 2016cited by 320position: middledoi
A Founder Mutation in VPS11 Causes an Autosomal Recessive Leukoencephalopathy Linked to Autophagic Defects
PLoS Genetics 2016cited by 71position: middledoi
Glutamine and hyperammonemic crises in patients with urea cycle disorders
Molecular Genetics and Metabolism 2015cited by 39position: middledoi
Blood ammonia and glutamine as predictors of hyperammonemic crises in patients with urea cycle disorder
Genetics in Medicine 2014cited by 41position: middledoi
Treatment-related signs and symptoms among patients with urea cycle disorders (UCDs) during treatment with sodium phenylbutyrate and glycerol phenylbutyrate
Clinical Biochemistry 2014cited by 0position: middledoi
Ammonia Control in Children Ages 2 Months through 5 Years with Urea Cycle Disorders: Comparison of Sodium Phenylbutyrate and Glycerol Phenylbutyrate
The Journal of Pediatrics 2013cited by 44position: middledoi
Elevated phenylacetic acid levels do not correlate with adverse events in patients with urea cycle disorders or hepatic encephalopathy and can be predicted based on the plasma PAA to PAGN ratio
Molecular Genetics and Metabolism 2013cited by 37position: middledoi
Ammonia control and neurocognitive outcome among urea cycle disorder patients treated with glycerol phenylbutyrate
Hepatology 2012cited by 95position: firstdoi
Urinary phenylacetylglutamine as dosing biomarker for patients with urea cycle disorders
Molecular Genetics and Metabolism 2012cited by 48position: middledoi

Grants

No grants ingested yet.

Frequent collaborators

Shawn E. McCandless · Children's Hospital Colorado4 papers (2013–2019) · 4 papers (2013–2019) · 4 papers (2021–2022)Cary O. Harding · Oregon Health & Science University4 papers (2013–2019)Uta Lichter‐Konecki · University of Pittsburgh4 papers (2013–2019) · 3 papers (2013–2015) · 3 papers (2013–2015) · 3 papers (2013–2015)Nicola Longo · Center for Human Genetics3 papers (2014–2019)Annette Feigenbaum · University of Toronto3 papers (2014–2019)Renata C. Gallagher · University of Colorado Denver3 papers (2014–2019)Susan A. Berry · University of Aberdeen3 papers (2013–2019)William J. Rhead · Medical College of Wisconsin3 papers (2014–2019)Andreas Schulze · Hospital for Sick Children3 papers (2014–2019)William Berquist · Palo Alto University2 papers (2015–2019) · 2 papers (2014–2015)J. Lawrence Merritt · Boston Medical Center1 papers (2019–2019) · 1 papers (2015–2015)Jerry Vockley · Pittsburgh Public Schools1 papers (2019–2019)Derek A. Wong · The University of Sydney1 papers (2019–2019)