World's Best Scientists 2026 revealed!

D-Index & Metrics

Biology and Biochemistry

D-Index
70
Citations
12810
World Ranking
7188
National Ranking
58

Overview

Wolfgang Sperl is affiliated with Paracelsus Medical University in Austria and has contributed extensively to the fields of biochemistry, genetics, molecular biology, and medicine. Their research primarily explores mitochondrial function and pathology, metabolism and genetic disorders, and child and adolescent health. This work intersects several subfields including molecular biology, clinical biochemistry, genetics, pediatrics, perinatology, and nutrition and dietetics.

The scientist's publication record encompasses a variety of topics centered on mitochondrial biology, metabolic diseases, and pediatric healthcare. Notable recent papers include:

  • Role of Energy Metabolism and Mitochondrial Function in Inflammatory Bowel Disease, 2022, published in Inflammatory Bowel Diseases
  • Age-Related Deterioration of Mitochondrial Function in the Intestine, 2020, published in Oxidative Medicine and Cellular Longevity
  • Austrian study shows that delays in accessing acute paediatric health care outweighed the risks of COVID-19, 2020, published in Acta Paediatrica
  • Expression of Oxidative Phosphorylation Complexes and Mitochondrial Mass in Pediatric and Adult Inflammatory Bowel Disease, 2022, published in Oxidative Medicine and Cellular Longevity
  • Glutaric Aciduria Type I Missed by Newborn Screening: Report of Four Cases from Three Families, 2021, published in International Journal of Neonatal Screening

The scientist frequently collaborates with other researchers in related fields, including Daniel Weghuber, Anna-Maria Schneider, Nadja Haiden, Saskia B. Wortmann, and Johannes A. Mayr. These collaborations have contributed to multi-authored works in pediatric and molecular biology research.

Wolfgang Sperl has published multiple articles in specialized journals such as Monatsschrift Kinderheilkunde, Pädiatrie & Pädologie, Oxidative Medicine and Cellular Longevity, International Journal of Neonatal Screening, and Inflammatory Bowel Diseases. Monatsschrift Kinderheilkunde accounts for a significant portion of the scientist's published work.

The research topics that are central to their work include:

  • Mitochondrial Function and Pathology
  • Metabolism and Genetic Disorders
  • Child and Adolescent Health
  • Diet and Metabolism Studies
  • Genomics and Rare Diseases
  • Adolescent and Pediatric Healthcare
  • ATP Synthase and ATPases Research

Overall, Wolfgang Sperl's body of work reflects a multidisciplinary approach targeting fundamental biochemical processes, clinical applications in pediatrics, and molecular mechanisms underlying metabolic and mitochondrial diseases.

Best Publications

  • Lipoic acid biosynthesis defects

    Johannes A. Mayr;René G. Feichtinger;Frederic Tort;Antonia Ribes

  • TMEM70 mutations cause isolated ATP synthase deficiency and neonatal mitochondrial encephalocardiomyopathy.

    Alena Cízková;Viktor Stránecký;Johannes A Mayr;Markéta Tesarová

  • Mitochondrial translation requires folate-dependent tRNA methylation

    Raphael J. Morscher;Gregory S. Ducker;Sophia Hsin Jung Li;Johannes A. Mayer

  • Lack of the mitochondrial protein acylglycerol kinase causes Sengers syndrome.

    Johannes A. Mayr;Tobias B. Haack;Elisabeth Graf;Franz A. Zimmermann

  • Molecular diagnosis in mitochondrial complex I deficiency using exome sequencing

    Tobias B Haack;Birgit Haberberger;Eva-Maria Frisch;Thomas Wieland

  • Mitochondria: The ketogenic diet--A metabolism-based therapy.

    Silvia Vidali;Sepideh Aminzadeh;Bridget Lambert;Tricia Rutherford

  • Propionic acidemia: clinical course and outcome in 55 pediatric and adolescent patients

    Sarah C. Grünert;Stephanie Müllerleile;Linda De Silva;Michael Barth

  • Inhibition of Neuroblastoma Tumor Growth by Ketogenic Diet and/or Calorie Restriction in a CD1-Nu Mouse Model

    Raphael Johannes Morscher;Sepideh Aminzadeh-Gohari;René Gunther Feichtinger;Johannes Adalbert Mayr

  • 2-Methyl-3-Hydroxybutyryl-CoA Dehydrogenase Deficiency Is Caused by Mutations in the HADH2 Gene

    Rob Ofman;Jos P. N. Ruiter;Marike Feenstra;Marinus Duran

  • Mitochondrial phosphate-carrier deficiency: a novel disorder of oxidative phosphorylation.

    Johannes A. Mayr;Olaf Merkel;Sepp D. Kohlwein;Boris R. Gebhardt

  • Decrease of mitochondrial DNA content and energy metabolism in renal cell carcinoma.

    David Meierhofer;David Meierhofer;Johannes A. Mayr;Ulrike Foetschl;Alexandra Berger

  • Loss of Complex I due to Mitochondrial DNA Mutations in Renal Oncocytoma

    Johannes A. Mayr;David Meierhofer;David Meierhofer;Franz Zimmermann;Rene Feichtinger

  • Mitochondrial ATP synthase deficiency due to a mutation in the ATP5E gene for the F1 ε subunit

    Johannes A. Mayr;Vendula Havlíčková;Franz Zimmermann;Iris Magler

  • Influence of hematocrit and localisation of punch in dried blood spots on levels of amino acids and acylcarnitines measured by tandem mass spectrometry.

    Margareta Holub;Karin Tuschl;Rene Ratschmann;Kristina Anna Strnadová

  • Mutations in FBXL4, Encoding a Mitochondrial Protein, Cause Early-Onset Mitochondrial Encephalomyopathy

    Xiaowu Gai;Daniele Ghezzi;Mark A. Johnson;Caroline A. Biagosch

  • Propionic acidaemia: clinical, biochemical and therapeutic aspects. Experience in 30 patients.

    W Lehnert;W Sperl;T Suormala;E R Baumgartner

  • Role of bacteria in the pathogenesis of short bowel syndrome-associated D-lactic acidemia.

    G.P..A Bongaerts;J.J.M. Tolboom;A.H.J. Naber;W.J.K. Sperl

  • Disturbed mitochondrial and peroxisomal dynamics due to loss of MFF causes Leigh-like encephalopathy, optic atrophy and peripheral neuropathy

    Johannes Koch;René G Feichtinger;Peter Freisinger;Mechthild Pies

  • A ketogenic diet supplemented with medium-chain triglycerides enhances the anti-tumor and anti-angiogenic efficacy of chemotherapy on neuroblastoma xenografts in a CD1-nu mouse model

    Sepideh Aminzadeh-Gohari;René Günther Feichtinger;Silvia Vidali;Felix Locker

  • Natural disease course and genotype-phenotype correlations in Complex I deficiency caused by nuclear gene defects: what we learned from 130 cases

    S. Koene;R.J.T. Rodenburg;M.S. van der Knaap;M.A.A.P. Willemsen

Frequent Co-Authors

Johannes A. Mayr
Johannes A. Mayr Paracelsus Medical University
Holger Prokisch
Holger Prokisch Technical University of Munich
Tobias B. Haack
Tobias B. Haack University of Tübingen
Thomas Meitinger
Thomas Meitinger Technical University of Munich
Tim M. Strom
Tim M. Strom Technical University of Munich
Richard J. Rodenburg
Richard J. Rodenburg Radboud University
Ilka Wittig
Ilka Wittig Goethe University Frankfurt
Thomas Klopstock
Thomas Klopstock Ludwig-Maximilians-Universität München
Robert W. Taylor
Robert W. Taylor Newcastle University
Massimo Zeviani
Massimo Zeviani University of Padua

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