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José E. Abdenur

Children’s Institute ·
Area of research
Clinical Biochemistry · Molecular Biology
Research interest
Research interests include Metabolism and Genetic Disorders, Mitochondrial Function and Pathology, Folate and B Vitamins Research, and Genomics and Rare Diseases.
h-index
35
citations
4,497
works
110
NIH funding
primary concept
email

Recent publications

Urine organic acid analysis as a tool in evaluation for Zellweger Spectrum disorder: A retrospective study
Molecular Genetics and Metabolism 2025cited by 0position: middledoi
Association Between Lysine Reduction Therapies and Cognitive Outcomes in Patients With Pyridoxine-Dependent Epilepsy
Neurology 2022cited by 41position: middledoi
Timing of therapy and neurodevelopmental outcomes in 18 families with pyridoxine-dependent epilepsy
Molecular Genetics and Metabolism 2022cited by 27position: middledoi
Consensus guidelines for the diagnosis and management of pyridoxine‐dependent epilepsy due to α‐aminoadipic semialdehyde dehydrogenase deficiency
Journal of Inherited Metabolic Disease 2020cited by 116position: middledoi
Treatment outcome of twenty-two patients with guanidinoacetate methyltransferase deficiency: An international retrospective cohort study
European Journal of Paediatric Neurology 2018cited by 34position: middledoi
Biochemical characteristics of newborns with carnitine transporter defect identified by newborn screening in California
Molecular Genetics and Metabolism 2017cited by 38position: lastdoi
An overview of combined D‐2‐ and L‐2‐hydroxyglutaric aciduria: functional analysis of CIC variants
Journal of Inherited Metabolic Disease 2017cited by 35position: middledoi
Activation of a cryptic splice site in the mitochondrial elongation factor GFM1 causes combined OXPHOS deficiency
Mitochondrion 2017cited by 24position: lastdoi
Mutations in TFAM, encoding mitochondrial transcription factor A, cause neonatal liver failure associated with mtDNA depletion
Molecular Genetics and Metabolism 2016cited by 121position: lastdoi
ALG1-CDG: Clinical and Molecular Characterization of 39 Unreported Patients
Human Mutation 2016cited by 56position: middledoi
Mudd’s disease (MAT I/III deficiency): a survey of data for MAT1A homozygotes and compound heterozygotes
Orphanet Journal of Rare Diseases 2015cited by 47position: middledoi
Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics
Genetics in Medicine 2014cited by 491position: middledoi
Mutations in GDP-Mannose Pyrophosphorylase B Cause Congenital and Limb-Girdle Muscular Dystrophies Associated with Hypoglycosylation of α-Dystroglycan
The American Journal of Human Genetics 2013cited by 239position: middledoi
Biochemical, molecular, and clinical characteristics of children with short chain acyl-CoA dehydrogenase deficiency detected by newborn screening in California
Molecular Genetics and Metabolism 2012cited by 74position: middledoi

Grants

No grants ingested yet.

Frequent collaborators

Monica Boyer · Children's Hospital of Orange County3 papers (2017–2022) · 2 papers (2012–2017) · 2 papers (2016–2017)Curtis R. Coughlin · University of Colorado Anschutz Medical Campus2 papers (2022–2022)Clara D.M. van Karnebeek · University of British Columbia2 papers (2022–2022)Nicola Longo · Center for Human Genetics2 papers (2022–2022)Ashley Andrews · Andrews University2 papers (2022–2022)Laura A. Tseng · Emma Kinderziekenhuis2 papers (2022–2022)Hans Hartmann · Medizinische Hochschule Hannover2 papers (2022–2022)Hao Tang · Chongqing University of Technology2 papers (2012–2017)Richard Chang · Southwestern Medical Center2 papers (2016–2017)Raymond Wang · Massachusetts Institute of Technology2 papers (2012–2017)Roelineke J. Lunsing · University Medical Center Groningen2 papers (2022–2022)Frits A. Wijburg · Emma Kinderziekenhuis2 papers (2022–2022)Emma Footitt · Great Ormond Street Hospital2 papers (2022–2022)Mirian C. H. Janssen · Radboud University Nijmegen2 papers (2022–2022) · 2 papers (2012–2017)Levinus A. Bok · Radboud University Nijmegen2 papers (2022–2022) · 2 papers (2012–2017)Natalie M. Gallant · Salford Royal Hospital2 papers (2012–2017)