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Ben Distel

Ben Distel

D-Index & Metrics

Biology and Biochemistry

D-Index
49
Citations
8491
World Ranking
18040
National Ranking
436

Overview

Ben Distel is affiliated with Erasmus MC in the Netherlands and has a research focus primarily in the fields of biochemistry, genetics, and molecular biology. Their work spans multiple subfields including genetics, molecular biology, cell biology, pediatrics, perinatology, child health, and surgery.

Their research addresses several topics, emphasizing genetics and neurodevelopmental disorders, genetic syndromes and imprinting, as well as epigenetics and DNA methylation. Additional areas of study include RNA modifications and cancer, cancer-related gene regulation, cellular transport and secretion, and congenital heart defects research.

Ben Distel has coauthored with several frequent collaborators, including:

  • Ype Elgersma
  • A. Mattijs Punt
  • F. Isabella Zampeta
  • Edwin Mientjes
  • Rob F. M. Verhagen

Their publications appear in a variety of scientific journals, with frequent contributions to the following venues:

  • Human Molecular Genetics
  • bioRxiv (Cold Spring Harbor Laboratory)
  • Human Mutation
  • Cell Reports Medicine
  • Molecular Psychiatry

Some recent papers authored or coauthored by Ben Distel include:

  • TAOK1 is associated with neurodevelopmental disorder and essential for neuronal maturation and cortical development, 2021, Human Mutation
  • Secreted retrovirus-like GAG-domain-containing protein PEG10 is regulated by UBE3A and is involved in Angelman syndrome pathophysiology, 2021, Cell Reports Medicine
  • Loss of nuclear UBE3A activity is the predominant cause of Angelman syndrome in individuals carrying UBE3A missense mutations, 2021, Human Molecular Genetics
  • A cross-species spatiotemporal proteomic analysis identifies UBE3A-dependent signaling pathways and targets, 2022, Molecular Psychiatry
  • Conserved UBE3A subcellular distribution between human and mice is facilitated by non-homologous isoforms, 2020, Human Molecular Genetics

Best Publications

  • The ABC transporter proteins Pat1 and Pat2 are required for import of long-chain fatty acids into peroxisomes of Saccharomyces cerevisiae.

    E. H. Hettema;C. W. T. Van Roermund;B. Distel;M. Van Den Berg

  • Peroxisomal protein import is conserved between yeast, plants, insects and mammals.

    Stephen J. Gould;Gilbert Andre Keller;Michel Schneider;Stephen H. Howell

  • Saccharomyces cerevisiae pex3p and pex19p are required for proper localization and stability of peroxisomal membrane proteins.

    Ewald H. Hettema;Wolfgang Girzalsky;Marlene van den Berg;Ralf Erdmann

  • Ube3a reinstatement identifies distinct developmental windows in a murine Angelman syndrome model

    Sara Silva-Santos;Geeske M. van Woerden;Caroline F. Bruinsma;Edwin Mientjes

  • Overexpression of Pex15p, a phosphorylated peroxisomal integral membrane protein required for peroxisome assembly in S.cerevisiae, causes proliferation of the endoplasmic reticulum membrane

    Y. Elgersma;L. Kwast;M. van den Berg;W.B. Snyder

  • Rhizomelic chondrodysplasia punctata is a peroxisomal protein targeting disease caused by a non-functional PTS2 receptor.

    A. M. Motley;E. H. Hettema;E. M. Hogenhout;P. Brites

  • PAS10 is a tetratricopeptide-repeat protein that is essential for the import of most matrix proteins into peroxisomes of Saccharomyces cerevisiae

    I Van der Leij;M M Franse;Y Elgersma;B Distel

  • The SH3 domain of the Saccharomyces cerevisiae peroxisomal membrane protein Pex13p functions as a docking site for Pex5p, a mobile receptor for the import PTS1-containing proteins.

    Y Elgersma;L Kwast;A Klein;T Voorn-Brouwer

  • A Conserved Cysteine Is Essential for Pex4p-dependent Ubiquitination of the Peroxisomal Import Receptor Pex5p

    Chris Williams;Marlene van den Berg;Richard R. Sprenger;Ben Distel

  • Involvement of the Endoplasmic Reticulum in Peroxisome Formation

    Hans J. Geuze;Jean Luc Murk;An K. Stroobants;Janice M. Griffith

  • Construction of cDNA coding for human von Willebrand factor using antibody probes for colony-screening and mapping of the chromosomal gene

    Cornelis L. Verweij;Carlie J.M. de Vries;Ben Distel;Anton-Jan van Zonneveld

  • Analysis of the Carboxyl-terminal Peroxisomal Targeting Signal 1 in a Homologous Context in Saccharomyces cerevisiae

    Ype Elgersma;Arnold Vos;Marlene van den Berg;Carlo W.T. van Roermund

  • De Novo Mutations in Protein Kinase Genes CAMK2A and CAMK2B Cause Intellectual Disability

    Sébastien Küry;Geeske M van Woerden;Thomas Besnard;Martina Proietti Onori

  • Saccharomyces cerevisiae acyl-CoA oxidase follows a novel, non-PTS1, import pathway into peroxisomes that is dependent on Pex5p

    André T.J. Klein;Marlene van den Berg;Gina Bottger;Henk F. Tabak

  • The Saccharomyces cerevisiae peroxisomal import receptor Pex5p is monoubiquitinated in wild type cells.

    Astrid Kragt;Tineke M Voorn-Brouwer;Marlene Van den Berg;Ben Distel

  • Enzymology of the carnitine biosynthesis pathway.

    Karin Strijbis;Frédéric M. Vaz;Ben Distel

  • Intracellular Acetyl Unit Transport in Fungal Carbon Metabolism

    Karin Strijbis;Ben Distel

  • Endoplasmic reticulum-directed Pex3p routes to peroxisomes and restores peroxisome formation in a Saccharomyces cerevisiae pex3Delta strain.

    Astrid Kragt;Tineke Voorn-Brouwer;Marlene van den Berg;Ben Distel

  • Isolation of peroxisome assembly mutants from Saccharomyces cerevisiae with different morphologies using a novel positive selection procedure.

    I. van der Leij;M. van den Berg;R.G. Boot;M. Franse

  • Regulating the human HECT E3 ligases

    Jasper Sluimer;Ben Distel;Ben Distel

Frequent Co-Authors

Henk F. Tabak
Henk F. Tabak University of Amsterdam
Ype Elgersma
Ype Elgersma Erasmus University Rotterdam
Matthias Wilmanns
Matthias Wilmanns Universität Hamburg
Marten Veenhuis
Marten Veenhuis University of Groningen
Ralf Erdmann
Ralf Erdmann Ruhr University Bochum
Michael Sattler
Michael Sattler Technical University of Munich
Ineke Braakman
Ineke Braakman Utrecht University
Donna M. MacCallum
Donna M. MacCallum University of Aberdeen
Andrew O.M. Wilkie
Andrew O.M. Wilkie University of Oxford
Frank Baas
Frank Baas Leiden University Medical Center

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