Insulin resistance and the metabolism of branched-chain amino acids in humans
MM Adeva, J Calviño, G Souto, C Donapetry - Amino acids, 2012 - Springer
Peripheral resistance to insulin action is the major mechanism causing the metabolic
syndrome and eventually type 2 diabetes mellitus. The metabolic derangement associated …
syndrome and eventually type 2 diabetes mellitus. The metabolic derangement associated …
A novel branched-chain amino acid metabolon: Protein-protein interactions in a supramolecular complex
MM Islam, R Wallin, RM Wynn, M Conway… - Journal of Biological …, 2007 - ASBMB
The catabolic pathways of branched-chain amino acids have two common steps. The first
step is deamination catalyzed by the vitamin B 6-dependent branched-chain …
step is deamination catalyzed by the vitamin B 6-dependent branched-chain …
Directed regulation of multienzyme complexes of 2-oxo acid dehydrogenases using phosphonate and phosphinate analogs of 2-oxo acids
AV Artiukhov, AV Graf, VI Bunik - Biochemistry (Moscow), 2016 - Springer
Oxo acid dehydrogenase complexes are important metabolic checkpoints functioning at the
intercept of sugar and amino acid degradation. This review presents a short summary of …
intercept of sugar and amino acid degradation. This review presents a short summary of …
Structural and thermodynamic basis for weak interactions between dihydrolipoamide dehydrogenase and subunit-binding domain of the branched-chain α-ketoacid …
CA Brautigam, RM Wynn, JL Chuang, MT Naik… - Journal of Biological …, 2011 - ASBMB
The purified mammalian branched-chain α-ketoacid dehydrogenase complex (BCKDC),
which catalyzes the oxidative decarboxylation of branched-chain α-keto acids, is essentially …
which catalyzes the oxidative decarboxylation of branched-chain α-keto acids, is essentially …
Molecular and structural analyses of maple syrup urine disease and identification of a founder mutation in a Portuguese Gypsy community
S Quental, S Macedo-Ribeiro, R Matos… - Molecular genetics and …, 2008 - Elsevier
Maple syrup urine disease (MSUD) is an autosomal recessive disorder, caused by the
defective function of the branched-chain α-ketoacid dehydrogenase complex (BCKD). BCKD …
defective function of the branched-chain α-ketoacid dehydrogenase complex (BCKD). BCKD …
NMR spectroscopy on domain dynamics in biomacromolecules
YE Shapiro - Progress in Biophysics and Molecular Biology, 2013 - Elsevier
Abstract Domain dynamics in biomacromolecules is currently an area of intense research
because of its importance for understanding the huge quantity of available data relating the …
because of its importance for understanding the huge quantity of available data relating the …
Molecular basis of the recognition of the ap65-1 gene transcription promoter elements by a Myb protein from the protozoan parasite Trichomonas vaginalis
Iron-inducible transcription of the ap65-1 gene in Trichomonas vaginalis involves at least
three Myb-like transcriptional factors (tv Myb1, tv Myb2 and tv Myb3) that differentially bind to …
three Myb-like transcriptional factors (tv Myb1, tv Myb2 and tv Myb3) that differentially bind to …
Distinct modes of recognition of the lipoyl domain as substrate by the E1 and E3 components of the pyruvate dehydrogenase multienzyme complex
M Fries, KM Stott, S Reynolds, RN Perham - Journal of molecular biology, 2007 - Elsevier
Two-dimensional 15N-heteronuclear single-quantum coherence (HSQC) NMR studies with
a di-domain (lipoyl domain+ linker+ peripheral subunit-binding domain) of the dihydrolipoyl …
a di-domain (lipoyl domain+ linker+ peripheral subunit-binding domain) of the dihydrolipoyl …
Nuclear magnetic resonance approaches in the study of 2-oxo acid dehydrogenase multienzyme complexes—a literature review
The 2-oxoacid dehydrogenase complexes (ODHc) consist of multiple copies of three
enzyme components: E1, a 2-oxoacid decarboxylase; E2, dihydrolipoyl acyl-transferase; …
enzyme components: E1, a 2-oxoacid decarboxylase; E2, dihydrolipoyl acyl-transferase; …
Four novel mutations identified in Norwegian patients result in intermittent maple syrup urine disease when combined with the R301C mutation
E Brodtkorb, J Strand, PH Backe, AM Lund… - Molecular genetics and …, 2010 - Elsevier
Maple syrup urine disease (MSUD) is caused by a defect in branched chain α-ketoacid
dehydrogenase complex (BCKD), an essential metabolon for the catabolism of the branched …
dehydrogenase complex (BCKD), an essential metabolon for the catabolism of the branched …