(Q41600458)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9308370%20AND%20SRC:MED&resulttype=core&format=json

6 November 2019

review article

1 reference

title

Impaired folding and subunit assembly as disease mechanism: the example of medium-chain acyl-CoA dehydrogenase deficiency (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9308370%20AND%20SRC:MED&resulttype=core&format=json

medium chain acyl-CoA dehydrogenase deficiency

6 November 2019

main subject

0 references

author name string

P Bross

series ordinal

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9308370%20AND%20SRC:MED&resulttype=core&format=json

6 November 2019

B S Andresen

series ordinal

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9308370%20AND%20SRC:MED&resulttype=core&format=json

6 November 2019

N Gregersen

series ordinal

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9308370%20AND%20SRC:MED&resulttype=core&format=json

6 November 2019

publication date

1 January 1998

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9308370%20AND%20SRC:MED&resulttype=core&format=json

Progress in Molecular Biology and Translational Science

6 November 2019

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9308370%20AND%20SRC:MED&resulttype=core&format=json

6 November 2019

volume

58

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9308370%20AND%20SRC:MED&resulttype=core&format=json

6 November 2019

page(s)

301-337

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9308370%20AND%20SRC:MED&resulttype=core&format=json

Molecular chaperones in cellular protein folding

6 November 2019

cites work

1 reference

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7 January 2021

Principles that govern the folding of protein chains

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Are there pathways for protein folding?

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

A determinable but unresolved problem.

1 reference

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7 January 2021

Finding the right fold

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The molten globule is a third thermodynamical state of protein molecules.

1 reference

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7 January 2021

Characterizing transition states in protein folding: an essential step in the puzzle

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Investigation of protein folding by mass spectrometry.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Optimization of rates of protein folding: the nucleation-condensation mechanism and its implications

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The general concept of molecular chaperones

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

HSP70 phylogeny and the relationship between archaebacteria, eubacteria, and eukaryotes.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Partner proteins determine multiple functions of Hsp70

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Peptide-binding specificity of the molecular chaperone BiP

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Different conformations for the same polypeptide bound to chaperones DnaK and GroEL.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Structural analysis of substrate binding by the molecular chaperone DnaK

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Evolution of the chaperonin families (Hsp60, Hsp10 and Tcp-1) of proteins and the origin of eukaryotic cells

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The crystal structure of the bacterial chaperonin GroEL at 2.8 A

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The 2.4 A crystal structure of the bacterial chaperonin GroEL complexed with ATP gamma S

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The crystal structure of the GroES co-chaperonin at 2.8 Å resolution

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Characterization of the active intermediate of a GroEL-GroES-mediated protein folding reaction

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Putting a lid on protein folding: structure and function of the co-chaperonin, GroES.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Chaperonin-facilitated protein folding: optimization of rate and yield by an iterative annealing mechanism

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The Clp proteins: proteolysis regulators or molecular chaperones?

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Hsp78, a Clp homologue within mitochondria, can substitute for chaperone functions of mt-hsp70

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

A molecular chaperone, ClpA, functions like DnaK and DnaJ.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Disassembly of the Mu transposase tetramer by the ClpX chaperone

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Human ClpP protease: cDNA sequence, tissue-specific expression and chromosomal assignment of the gene

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Mitochondrial molecular chaperones: their role in protein translocation

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The protein import machinery of the mitochondrial inner membrane

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The role of Hsp70 in conferring unidirectionality on protein translocation into mitochondria

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Dynamic interaction between Isp45 and mitochondrial hsp70 in the protein import system of the yeast mitochondrial inner membrane

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

A quantitative assessment of the role of the chaperonin proteins in protein folding in vivo.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Folding in vivo of bacterial cytoplasmic proteins: role of GroEL.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Mitochondrial heat-shock protein hsp60 is essential for assembly of proteins imported into yeast mitochondria

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Decreased synthesis and inefficient mitochondrial import of hsp60 in a patient with a mitochondrial encephalomyopathy

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

A fatal, systemic mitochondrial disease with decreased mitochondrial enzyme activities, abnormal ultrastructure of the mitochondria and deficiency of heat shock protein 60.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Cyclophilin catalyzes protein folding in yeast mitochondria

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Cyclophilin 20 is involved in mitochondrial protein folding in cooperation with molecular chaperones Hsp70 and Hsp60

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Thermolabile folding intermediates: inclusion body precursors and chaperonin substrates.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Amino acid substitutions influencing intracellular protein folding pathways

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Global suppression of protein folding defects and inclusion body formation

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Structural and genetic analysis of the folding and function of T4 lysozyme

1 reference

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7 January 2021

beta-oxidation of fatty acids in mitochondria, peroxisomes, and bacteria: a century of continued progress

1 reference

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7 January 2021

Relationship between carbohydrate and lipid metabolism and the energy balance of heart muscle

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Structure and mechanism of action of the acyl-CoA dehydrogenases

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Molecular evolution and substrate specificity of acyl-CoA dehydrogenases: chimaeric "medium/long' chain-specific enzyme from medium-chain acyl-CoA dehydrogenase

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

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.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Crystal structures of medium-chain acyl-CoA dehydrogenase from pig liver mitochondria with and without substrate

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

2-octynoyl coenzyme A is a mechanism-based inhibitor of pig kidney medium-chain acyl coenzyme A dehydrogenase: isolation of the target peptide

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Suberylglycine excretion in the urine from a patient with dicarboxylic aciduria

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

In vitro fibroblast studies in a patient with C6-C10-dicarboxylic aciduria: evidence for a defect in general acyl-CoA dehydrogenase

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Dicarboxylic aciduria: deficient [1-14C]octanoate oxidation and medium-chain acyl-CoA dehydrogenase in fibroblasts

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Medium-chain acyl-CoA dehydrogenase deficiency in children with non-ketotic hypoglycemia and low carnitine levels

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Medium-chain acyl-coenzyme A dehydrogenase deficiency: clinical course in 120 affected children.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Molecular genetic characterization and urinary excretion pattern of metabolites in two families with MCAD deficiency due to compound heterozygosity with a 13 base pair insertion in one allele

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Nucleotide sequence of medium-chain acyl-CoA dehydrogenase mRNA and its expression in enzyme-deficient human tissue

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Molecular basis of medium chain acyl-coenzyme A dehydrogenase deficiency. An A to G transition at position 985 that causes a lysine-304 to glutamate substitution in the mature protein is the single prevalent mutation

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Identification of a common mutation in patients with medium-chain acyl-CoA dehydrogenase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Molecular characterization of inherited medium-chain acyl-CoA dehydrogenase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Prevalence of K329E mutation in medium-chain acyl-CoA dehydrogenase gene determined from Guthrie cards

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Mutations in the medium chain acyl-CoA dehydrogenase (MCAD) gene

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Medium chain acyl-CoA dehydrogenase deficiency caused by a deletion of exons 11 and 12

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

A novel mutation in medium chain acyl-CoA dehydrogenase causes sudden neonatal death

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Medium chain acyl-CoA dehydrogenase deficiency in Pennsylvania: neonatal screening shows high incidence and unexpected mutation frequencies

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Rhabdomyolysis and acute encephalopathy in late onset medium chain acyl-CoA dehydrogenase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Expression and characterization of human mutant (glutamic acid304) medium-chain acyl-coenzyme A dehydrogenase in mammalian cells

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Biosynthesis of variant medium chain acyl-CoA dehydrogenase in cultured fibroblasts from patients with medium chain acyl-CoA dehydrogenase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Immunoreactive enzyme protein in medium-chain acyl-CoA dehydrogenase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Immunochemical characterization of variant medium-chain acyl-CoA dehydrogenase in fibroblasts from patients with medium-chain acyl-CoA dehydrogenase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

GroE heat-shock proteins promote assembly of foreign prokaryotic ribulose bisphosphate carboxylase oligomers in Escherichia coli.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Biosynthesis of four rat liver mitochondrial acyl-CoA dehydrogenases: in vitro synthesis, import into mitochondria, and processing of their precursors in a cell-free system and in cultured cells

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

DNA repair protein XPA binds replication protein A (RPA).

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Effects of two mutations detected in medium chain acyl-CoA dehydrogenase (MCAD)-deficient patients on folding, oligomer assembly, and stability of MCAD enzyme

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Folding and association of proteins

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Comparison between medium-chain acyl-CoA dehydrogenase mutant proteins overexpressed in bacterial and mammalian cells.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Identification of two variant short chain acyl-coenzyme A dehydrogenase alleles, each containing a different point mutation in a patient with short chain acyl-coenzyme A dehydrogenase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Evolution of HSP70 gene and its implications regarding relationships between archaebacteria, eubacteria, and eukaryotes

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Molecular evolution of the HSP70 multigene family

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The mutational spectrum in very long-chain acyl-CoA dehydrogenase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Defective protein folding as a basis of human disease

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Defective protein folding as a cause of disease

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Folding intermediates are involved in genetic diseases?

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The Z type variation of human alpha 1-antitrypsin causes a protein folding defect

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Interaction of ascorbate and alpha-tocopherol in resealed human erythrocyte ghosts. Transmembrane electron transfer and protection from lipid peroxidation

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

A recombinant peptide model of the first nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator: comparison of wild-type and delta F508 mutant forms

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The cystic fibrosis mutation (delta F508) does not influence the chloride channel activity of CFTR.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Retinitis pigmentosa: unfolding its mystery

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Protein oligomerization in the endoplasmic reticulum

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Ubiquitin and the enigma of intracellular protein degradation.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Batten disease and mitochondrial pathways of proteolysis

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The first characterization of a eubacterial proteasome: the 20S complex of Rhodococcus.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

New insights into proteasome function: from archaebacteria to drug development

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Selective protein degradation: a journey's end within the proteasome

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

The mechanism and functions of ATP-dependent proteases in bacterial and animal cells

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

A human mitochondrial ATP-dependent protease that is highly homologous to bacterial Lon protease

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

ATP-dependent protease La (lon) from Escherichia coli.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Homology in structural organization between E. coli ClpAP protease and the eukaryotic 26 S proteasome

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

Prolyl isomerases: role in protein folding.

1 reference

https://api.crossref.org/works/10.1016%2FS0079-6603%2808%2960040-9

7 January 2021

10.1016/S0079-6603(08)60040-9

Identifiers

DOI

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9308370%20AND%20SRC:MED&resulttype=core&format=json

6 November 2019

1 reference