Peter Bross

Overview

Best Publications

• α-cardiac actin is a novel disease gene in familial hypertrophic cardiomyopathy

  Jens Mogensen;Ib C. Klausen;Anders K. Pedersen;Henrik Egeblad

  508
  Citations

• Hereditary spastic paraplegia SPG13 is associated with a mutation in the gene encoding the mitochondrial chaperonin Hsp60.

  Jens Jacob Hansen;Alexandra Dürr;Isabelle Cournu-Rebeix;Costa Georgopoulos

  451
  Citations

• Protein Misfolding and Human Disease

  Niels Gregersen;Peter Gerd Bross;Søren Vang;Jane Hvarregaard Christensen

  389
  Citations

• Clear Correlation of Genotype with Disease Phenotype in Very–Long-Chain Acyl-CoA Dehydrogenase Deficiency

  Brage Storstein Andresen;Brage Storstein Andresen;Simon Olpin;Ben J.H.M. Poorthuis;Hans R. Scholte

  336
  Citations

• Medium-chain acyl-CoA dehydrogenase (MCAD) mutations identified by MS/MS-based prospective screening of newborns differ from those observed in patients with clinical symptoms: identification and characterization of a new, prevalent mutation that results in mild MCAD deficiency.

  Brage Storstein Andresen;Brage Storstein Andresen;Steve F. Dobrowolski;Linda O'Reilly;Joseph Muenzer

  323
  Citations

• Protein misfolding and degradation in genetic diseases

  Peter Bross;Thomas Juhl Corydon;Brage S. Andresen;Brage S. Andresen;Malene Munk Jørgensen

  303
  Citations

• Mitochondrial Hsp60 Chaperonopathy Causes an Autosomal-Recessive Neurodegenerative Disorder Linked to Brain Hypomyelination and Leukodystrophy

  Daniella Magen;Costa Georgopoulos;Peter Bross;Debbie Ang

  253
  Citations

• Clear relationship between ETF/ETFDH genotype and phenotype in patients with multiple acyl-CoA dehydrogenation deficiency.

  Rikke K.J. Olsen;Brage S. Andresen;Brage S. Andresen;Ernst Christensen;Peter Bross

  246
  Citations

• Mutation analysis in mitochondrial fatty acid oxidation defects: Exemplified by acyl-CoA dehydrogenase deficiencies, with special focus on genotype-phenotype relationship.

  Niels Gregersen;Brage S. Andresen;Brage S. Andresen;Morten J. Corydon;Thomas J. Corydon

  220
  Citations

• Genomic structure of the human mitochondrial chaperonin genes: HSP60 and HSP10 are localised head to head on chromosome 2 separated by a bidirectional promoter

  Jens J Hansen;Peter Bross;Majken Westergaard;Marit Nyholm Nielsen

  206
  Citations

• Dissection of functional domains in phage fd adsorption protein: discrimination between attachment and penetration sites

  Irina Stengele;Peter Bross;Xaver Garces;Jeanette Giray

  175
  Citations

• Protein Misfolding and Cellular Stress: An Overview

  Niels Gregersen;Niels Gregersen;Peter Bross;Peter Bross

  173
  Citations

• Protein Misfolding and Disease

  Peter Bross;Niels Gregersen

  172
  Citations

• Identification of Four New Mutations in the Short-Chain Acyl-CoA Dehydrogenase (SCAD) Gene in Two Patients: One of the Variant Alleles, 511C→T, is Present at an Unexpectedly High Frequency in the General Population, as Was the Case for 625G→A, Together Conferring Susceptibility to Ethylmalonic Aciduria

  Niels Gregersen;Vibeke S. Winter;Morten J. Corydon;Thomas J. Corydon

  171
  Citations

• Mitochondrial fatty acid oxidation defects—remaining challenges

  Niels Gregersen;Brage S. Andresen;Christina B. Pedersen;Rikke K. J. Olsen

  162
  Citations

• Cloning and Characterization of Human Very-Long-Chain Acyl-CoA Dehydrogenase cDNA, Chromosomal Assignment of the Gene and Identification in Four Patients of Nine Different Mutations Within the VLCAD Gene

  Brage Storstein Andresen;Peter Bross;Christine Vianey-Saban;Priscille Divry

  156
  Citations

• The molecular basis of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in compound heterozygous patients: is there correlation between genotype and phenotype?

  Brage Storstein Andresen;Peter Bross;Szabolcs Udvari;Szabolcs Udvari;Jean Kirk

  148
  Citations

• Leptin regulation of Hsp60 impacts hypothalamic insulin signaling.

  André Kleinridders;Hans P.M.M. Lauritzen;Siegfried Ussar;Jane H. Christensen

  139
  Citations

• The ACADS gene variation spectrum in 114 patients with short-chain acyl-CoA dehydrogenase (SCAD) deficiency is dominated by missense variations leading to protein misfolding at the cellular level

  Christina B. Pedersen;Steen Kølvraa;Agnete Kølvraa;Vibeke Stenbroen

  136
  Citations

• The Metabolic and Molecular Basis of Inherited Disease: Protein Folding and Misfolding: the Role of Cellular Protein Quality Control Systems in Inherited Disorders

  Peter Bross;Malene Munk Jørgensen;Niels Gregersen

  1
  Citations