The domain-specific and temperature-dependent protein misfolding phenotype of variant medium-chain acyl-CoA dehydrogenase

PLoS ONE

Volumn 9, Issue 4, 2014, Pages

  Jank, Johanna M ^a   Maier, Esther M ^a,e   Reib, Dunja D ^a   Haslbeck, Martin ^b   Kemter, Kristina F ^a,f   Truger, Marietta S ^a,g   Sommerhoff, Christian P ^c   Ferdinandusse, Sacha ^d   Wanders, Ronald J ^d   Gersting, Søren W ^a   Muntau, Ania C ^a  

^a LUDWIG MAXIMILIANS UNIVERSITY   (Germany)

^b TECHNICAL UNIVERSITY OF MUNICH   (Germany)

^c UNIVERSITY OF MUNICH   (Germany)

^d UNIVERSITY OF AMSTERDAM   (Netherlands)

^e UNIVERSITY HOSPITAL INNSBRUCK   (Austria)

^f LEUKOCARE AG   (Germany)

^g MLL MUNICH LEUKEMIA LABORATORY   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

ALANINE; ARGININE; ASPARAGINE; CYSTEINE; ENZYME VARIANT; GLUTAMIC ACID; GLYCINE; HISTIDINE; LYSINE; MEDIUM CHAIN ACYL COENZYME A DEHYDROGENASE; SERINE; TYROSINE; VALINE; ACYL COENZYME A DEHYDROGENASE; FLAVINE ADENINE NUCLEOTIDE; MUTANT PROTEIN; PROTEIN AGGREGATE;

ACADM GENE; ANIMAL CELL; ARTICLE; CONTROLLED STUDY; DISEASE SEVERITY; ENZYME ACTIVITY; ENZYME CONFORMATION; ENZYME KINETICS; ENZYME SPECIFICITY; ENZYME STABILITY; GENE; GENE STRUCTURE; HIGH TEMPERATURE; MEDIUM CHAIN ACYL COENZYME A DEHYDROGENASE DEFICIENCY; METABOLIC DISORDER; MISSENSE MUTATION; MUTATIONAL ANALYSIS; NONHUMAN; NUCLEOTIDE SEQUENCE; OBSERVATIONAL STUDY; PHENOTYPE; PROTEIN DOMAIN; PROTEIN FOLDING; RISK FACTOR; STRUCTURE ANALYSIS; TEMPERATURE DEPENDENCE; TEMPERATURE STRESS; THERMOSTABILITY; ANIMAL; CHEMICAL PHENOMENA; CHEMICAL STRUCTURE; CHEMISTRY; CHLOROCEBUS AETHIOPS; CIRCULAR DICHROISM; COS 1 CELL LINE; ENZYME ACTIVATION; FLUORESCENCE; GENETICS; HEAT; HUMAN; KINETICS; METABOLISM; PROTEIN DENATURATION; PROTEIN SECONDARY STRUCTURE; PROTEIN TERTIARY STRUCTURE; TEMPERATURE;

ACYL-COA DEHYDROGENASE; ANIMALS; CERCOPITHECUS AETHIOPS; CIRCULAR DICHROISM; COS CELLS; ENZYME ACTIVATION; FLAVIN-ADENINE DINUCLEOTIDE; FLUORESCENCE; HOT TEMPERATURE; HUMANS; HYDROPHOBIC AND HYDROPHILIC INTERACTIONS; KINETICS; MODELS, MOLECULAR; MUTANT PROTEINS; MUTATION, MISSENSE; PHENOTYPE; PROTEIN AGGREGATES; PROTEIN DENATURATION; PROTEIN FOLDING; PROTEIN STRUCTURE, SECONDARY; PROTEIN STRUCTURE, TERTIARY; TEMPERATURE;

EID: 84899517298     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0093852     Document Type: Article

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