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Matthias Gautschi

University Hospital of Bern ·
Area of research
Clinical Biochemistry · Physiology
Research interest
Research interests include Metabolism and Genetic Disorders, Lysosomal Storage Disorders Research, Mitochondrial Function and Pathology, and Glycogen Storage Diseases and Myoclonus.
h-index
28
citations
4,201
works
101
NIH funding
primary concept
email

Recent publications

Long-term efficacy and safety of arimoclomol in Niemann-Pick disease type C: Final results of the phase 2/3 NPC-002 48-month open-label extension trial
Molecular Genetics and Metabolism 2025cited by 1position: middledoi
Assessment of the reliability, responsiveness, and meaningfulness of the scale for the assessment and rating of ataxia (SARA) for lysosomal storage disorders
Journal of Neurology 2024cited by 8position: middledoi
The natural history of classic galactosemia: lessons from the GalNet registry
Orphanet Journal of Rare Diseases 2019cited by 147position: middledoi
Epidemiology of mucopolysaccharidoses
Molecular Genetics and Metabolism 2017cited by 1,099position: middledoi
Fertility in adult women with classic galactosemia and primary ovarian insufficiency
Fertility and Sterility 2017cited by 61position: middledoi
International clinical guideline for the management of classical galactosemia: diagnosis, treatment, and follow‐up
Journal of Inherited Metabolic Disease 2016cited by 194position: middledoi
Frequency and Pathophysiology of Acute Liver Failure in Ornithine Transcarbamylase Deficiency (OTCD)
PLoS ONE 2016cited by 52position: middledoi
Cross-sectional study of 168 patients with hepatorenal tyrosinaemia and implications for clinical practice
Orphanet Journal of Rare Diseases 2014cited by 135position: middledoi
Cross‐sectional observational study of 208 patients with non‐classical urea cycle disorders
Journal of Inherited Metabolic Disease 2013cited by 79position: middledoi

Grants

No grants ingested yet.

Frequent collaborators

· 2 papers (2016–2017)Cynthia S. Gubbels · Massachusetts Institute of Technology2 papers (2016–2017) · 2 papers (2016–2017) · 2 papers (2016–2017) · 2 papers (2016–2025)Gerard T. Berry · Boston Children's Hospital2 papers (2016–2017) · 2 papers (2017–2024)Katrin Õunap · Tartu University Hospital2 papers (2016–2017) · 1 papers (2016–2016)Shaukat Khan · Thomas Jefferson University1 papers (2017–2017) · 1 papers (2017–2017)Shunji Tomatsu · Thomas Jefferson University1 papers (2017–2017) · 1 papers (2025–2025)Susanne A. Schneider · Johannes Gutenberg University Mainz1 papers (2024–2024)Janneke G. Langendonk · Erasmus MC1 papers (2017–2017) · 1 papers (2025–2025) · 1 papers (2017–2017)Paul Gissen · University of Birmingham1 papers (2024–2024) · 1 papers (2025–2025)Siyamini Sivananthan · Cambridge University Hospitals NHS Foundation Trust1 papers (2024–2024)