List of works - Peter Bross

Disease-Associated Mutations in the HSPD1 Gene Encoding the Large Subunit of the Mitochondrial HSP60/HSP10 Chaperonin Complex

scientific article

Single-nucleotide variations in the genes encoding the mitochondrial Hsp60/Hsp10 chaperone system and their disease-causing potential

article

Release of periplasmic proteins induced in E. coli by expression of an N-terminal proximal segment of the phage fd gene 3 protein

scientific article published in March 1991

A cell model to study different degrees of Hsp60 deficiency in HEK293 cells.

scientific article published on 30 June 2011

Expression of transforming growth factor alpha and epidermal growth factor receptor in human bladder cancer

scientific article published on 01 July 1999

The clinical outcome of LMNA missense mutations can be associated with the amount of mutated protein in the nuclear envelope

scientific article published on 26 June 2018

Do lamin A and lamin C have unique roles?

scientific article published on 07 October 2014

Differential degradation of variant medium-chain acyl-CoA dehydrogenase by the protein quality control proteases Lon and ClpXP.

scientific article

Characterization of wild-type human medium-chain acyl-CoA dehydrogenase (MCAD) and mutant enzymes present in MCAD-deficient patients by two-dimensional gel electrophoresis: evidence for post-translational modification of the enzyme

scientific article published on 01 June 1994

The LMNA mutation p.Arg321Ter associated with dilated cardiomyopathy leads to reduced expression and a skewed ratio of lamin A and lamin C proteins

scientific article published on 31 August 2013

Mitochondrial Spare Respiratory Capacity Is Negatively Correlated with Nuclear Reprogramming Efficiency.

scientific article published on 26 October 2016

Selected reaction monitoring as an effective method for reliable quantification of disease-associated proteins in maple syrup urine disease.

scientific article

Characterization of a disease-causing Lys329 to Glu mutation in 16 patients with medium-chain Acyl-CoA dehydrogenase deficiency

article

A polymorphic position in electron transfer flavoprotein modulates kinetic stability as evidenced by thermal stress.

scientific article published on 8 January 2011

Heterozygosity for an in-frame deletion causes glutaryl-CoA dehydrogenase deficiency in a patient detected by newborn screening: investigation of the effect of the mutant allele.

scientific article

Metformin targets brown adipose tissue in vivo and reduces oxygen consumption in vitro

scientific article published on 08 June 2018

Glycosylation of the N-terminal potential N-glycosylation sites in the human alpha1,3-fucosyltransferase V and -VI (hFucTV and -VI)

scientific article published on 01 December 2000

Prenatal diagnosis of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in a family with a previous fatal case of sudden unexpected death in childhood.

scientific article published on 01 January 1995

Application of an Image Cytometry Protocol for Cellular and Mitochondrial Phenotyping on Fibroblasts from Patients with Inherited Disorders

scientific article published on 25 September 2015

Deficiency of the mitochondrial sulfide regulator ETHE1 disturbs cell growth, glutathione level and causes proteome alterations outside mitochondria

scientific article published on 02 November 2018

Oxidative stress-induced metabolic changes in mouse C2C12 myotubes studied with high-resolution 13C, 1H, and 31P NMR spectroscopy.

scientific article

Metabolic profiling of heat or anoxic stress in mouse C2C12 myotubes using multinuclear magnetic resonance spectroscopy.

scientific article

Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency due to heterozygosity for the common mutation and an allele resulting in low levels of MCAD mRNA

article

An inventory of interactors of the human HSP60/HSP10 chaperonin in the mitochondrial matrix space

scientific article published on 14 February 2020

A Cell Model for HSP60 Deficiencies: Modeling Different Levels of Chaperonopathies Leading to Oxidative Stress and Mitochondrial Dysfunction

scientific article published on 01 January 2019

Molecular diagnosis and characterization of medium-chain acyl-CoA dehydrogenase deficiency

scientific article published on January 1, 1995

Riboflavin Deficiency-Implications for General Human Health and Inborn Errors of Metabolism

scientific article published on 28 May 2020

Potential complementation effects of two disease-associated mutations in tetrameric glutaryl-CoA dehydrogenase is due to inter subunit stability-activity counterbalance

scientific article published on 03 September 2019

Mitochondrial Hsp70 and the troubles of nomenclature: leaving behind tradition to gain intuitiveness and clarity

scientific article published on 22 May 2016

APD-Containing Cyclolipodepsipeptides Target Mitochondrial Function in Hypoxic Cancer Cells

scientific article published on 16 August 2018

Human skeletal muscle CD90⁺ fibro-adipogenic progenitors are associated with muscle degeneration in type 2 diabetic patients

scientific article published on 21 October 2021

Electron transfer flavoprotein and its role in mitochondrial energy metabolism in health and disease

scientific article

A recurrent de novo HSPD1 variant is associated with hypomyelinating leukodystrophy

scientific article published on 12 June 2020

List of works by Peter Bross

Disease-Associated Mutations in the HSPD1 Gene Encoding the Large Subunit of the Mitochondrial HSP60/HSP10 Chaperonin Complex

Single-nucleotide variations in the genes encoding the mitochondrial Hsp60/Hsp10 chaperone system and their disease-causing potential

Release of periplasmic proteins induced in E. coli by expression of an N-terminal proximal segment of the phage fd gene 3 protein

A cell model to study different degrees of Hsp60 deficiency in HEK293 cells.

Expression of transforming growth factor alpha and epidermal growth factor receptor in human bladder cancer

The clinical outcome of LMNA missense mutations can be associated with the amount of mutated protein in the nuclear envelope

Do lamin A and lamin C have unique roles?

Differential degradation of variant medium-chain acyl-CoA dehydrogenase by the protein quality control proteases Lon and ClpXP.

Characterization of wild-type human medium-chain acyl-CoA dehydrogenase (MCAD) and mutant enzymes present in MCAD-deficient patients by two-dimensional gel electrophoresis: evidence for post-translational modification of the enzyme

The LMNA mutation p.Arg321Ter associated with dilated cardiomyopathy leads to reduced expression and a skewed ratio of lamin A and lamin C proteins

Mitochondrial Spare Respiratory Capacity Is Negatively Correlated with Nuclear Reprogramming Efficiency.

Selected reaction monitoring as an effective method for reliable quantification of disease-associated proteins in maple syrup urine disease.

Characterization of a disease-causing Lys329 to Glu mutation in 16 patients with medium-chain Acyl-CoA dehydrogenase deficiency

A polymorphic position in electron transfer flavoprotein modulates kinetic stability as evidenced by thermal stress.

Heterozygosity for an in-frame deletion causes glutaryl-CoA dehydrogenase deficiency in a patient detected by newborn screening: investigation of the effect of the mutant allele.

Metformin targets brown adipose tissue in vivo and reduces oxygen consumption in vitro

Glycosylation of the N-terminal potential N-glycosylation sites in the human alpha1,3-fucosyltransferase V and -VI (hFucTV and -VI)

Prenatal diagnosis of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in a family with a previous fatal case of sudden unexpected death in childhood.

Application of an Image Cytometry Protocol for Cellular and Mitochondrial Phenotyping on Fibroblasts from Patients with Inherited Disorders

Deficiency of the mitochondrial sulfide regulator ETHE1 disturbs cell growth, glutathione level and causes proteome alterations outside mitochondria

Oxidative stress-induced metabolic changes in mouse C2C12 myotubes studied with high-resolution 13C, 1H, and 31P NMR spectroscopy.

Metabolic profiling of heat or anoxic stress in mouse C2C12 myotubes using multinuclear magnetic resonance spectroscopy.

Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency due to heterozygosity for the common mutation and an allele resulting in low levels of MCAD mRNA

An inventory of interactors of the human HSP60/HSP10 chaperonin in the mitochondrial matrix space

A Cell Model for HSP60 Deficiencies: Modeling Different Levels of Chaperonopathies Leading to Oxidative Stress and Mitochondrial Dysfunction

Molecular diagnosis and characterization of medium-chain acyl-CoA dehydrogenase deficiency

Riboflavin Deficiency-Implications for General Human Health and Inborn Errors of Metabolism

Potential complementation effects of two disease-associated mutations in tetrameric glutaryl-CoA dehydrogenase is due to inter subunit stability-activity counterbalance

Mitochondrial Hsp70 and the troubles of nomenclature: leaving behind tradition to gain intuitiveness and clarity

APD-Containing Cyclolipodepsipeptides Target Mitochondrial Function in Hypoxic Cancer Cells

Human skeletal muscle CD90⁺ fibro-adipogenic progenitors are associated with muscle degeneration in type 2 diabetic patients

Electron transfer flavoprotein and its role in mitochondrial energy metabolism in health and disease

A recurrent de novo HSPD1 variant is associated with hypomyelinating leukodystrophy