Pages that link to "Q22253419"
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The following pages link to Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease (Q22253419):
Displaying 50 items.
- 3-methyl-2-oxobutanoate dehydrogenase (lipoamide) complex (Q22327132) (← links)
- Solution structure and dynamics of the lipoic acid-bearing domain of human mitochondrial branched-chain alpha-keto acid dehydrogenase complex (Q24292283) (← links)
- Cross-talk between thiamin diphosphate binding and phosphorylation loop conformation in human branched-chain alpha-keto acid decarboxylase/dehydrogenase (Q24294487) (← links)
- Binding of pyruvate dehydrogenase to the core of the human pyruvate dehydrogenase complex (Q24307378) (← links)
- Roles of His291-alpha and His146-beta' in the reductive acylation reaction catalyzed by human branched-chain alpha-ketoacid dehydrogenase: refined phosphorylation loop structure in the active site (Q24313928) (← links)
- Molecular mechanism for regulation of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex by phosphorylation (Q24318778) (← links)
- Maple syrup urine disease: identification and carrier-frequency determination of a novel founder mutation in the Ashkenazi Jewish population (Q24533417) (← links)
- Milk--A Nutrient System of Mammalian Evolution Promoting mTORC1-Dependent Translation (Q26801523) (← links)
- The pathogenic role of persistent milk signaling in mTORC1- and milk-microRNA-driven type 2 diabetes mellitus (Q27027262) (← links)
- Structures of the human pyruvate dehydrogenase complex cores: a highly conserved catalytic center with flexible N-terminal domains (Q27303634) (← links)
- The crystal structure of yeast thiamin pyrophosphokinase (Q27632974) (← links)
- Structural basis for flip-flop action of thiamin pyrophosphate-dependent enzymes revealed by human pyruvate dehydrogenase (Q27640758) (← links)
- The two active sites in human branched-chain alpha-keto acid dehydrogenase operate independently without an obligatory alternating-site mechanism (Q27643936) (← links)
- A dual conformation of the post-decarboxylation intermediate is associated with distinct enzyme states in mycobacterial KGD (α-ketoglutarate decarboxylase) (Q27687388) (← links)
- Gene preference in maple syrup urine disease (Q28345008) (← links)
- BCKDH: the missing link in apicomplexan mitochondrial metabolism is required for full virulence of Toxoplasma gondii and Plasmodium berghei (Q28540877) (← links)
- Novel isoenzyme of 2-oxoglutarate dehydrogenase is identified in brain, but not in heart (Q29396800) (← links)
- Human microRNA (miR29b) expression controls the amount of branched chain alpha-ketoacid dehydrogenase complex in a cell. (Q30351534) (← links)
- Structural basis for activation of the thiamin diphosphate-dependent enzyme oxalyl-CoA decarboxylase by adenosine diphosphate. (Q30351586) (← links)
- Functional anthology of intrinsic disorder. 3. Ligands, post-translational modifications, and diseases associated with intrinsically disordered proteins. (Q30360723) (← links)
- Roles of active site and novel K ion-binding site residues in human mitochondrial branched-chain alpha-ketoacid decarboxylase/dehydrogenase (Q31538248) (← links)
- Serum Metabolomic Profiling of Piglets Infected with Virulent Classical Swine Fever Virus (Q33606965) (← links)
- Probing the mechanism of inactivation of human pyruvate dehydrogenase by phosphorylation of three sites (Q33926252) (← links)
- Maple syrup urine disease in Cypriot families: identification of three novel mutations and biochemical characterization of the p.Thr211Met mutation in the E1alpha subunit (Q34190871) (← links)
- Molecular architecture and mechanism of an icosahedral pyruvate dehydrogenase complex: a multifunctional catalytic machine (Q34206769) (← links)
- Distinct regulatory properties of pyruvate dehydrogenase kinase and phosphatase isoforms (Q34408068) (← links)
- Phenylbutyrate therapy for maple syrup urine disease (Q34505609) (← links)
- Asp295 Stabilizes the Active-Site Loop Structure of Pyruvate Dehydrogenase, Facilitating Phosphorylation of Ser292 by Pyruvate Dehydrogenase-Kinase (Q34557385) (← links)
- A structural perspective on enzymes activated by monovalent cations (Q36305162) (← links)
- Milk: an epigenetic amplifier of FTO-mediated transcription? Implications for Western diseases (Q36393233) (← links)
- Role of Na and K in enzyme function (Q36610450) (← links)
- Nuclear magnetic resonance approaches in the study of 2-oxo acid dehydrogenase multienzyme complexes--a literature review. (Q38146650) (← links)
- Thermodynamic analysis of the binding of component enzymes in the assembly of the pyruvate dehydrogenase multienzyme complex of Bacillus stearothermophilus. (Q38269524) (← links)
- Inhibition of the Escherichia coli pyruvate dehydrogenase complex E1 subunit and its tyrosine 177 variants by thiamin 2-thiazolone and thiamin 2-thiothiazolone diphosphates. Evidence for reversible tight-binding inhibition (Q38296259) (← links)
- Molecular Mechanisms of Enzyme Activation by Monovalent Cations. (Q38909804) (← links)
- Functional characterization of the novel intronic nucleotide change c.288 9C>T within the BCKDHA gene: understanding a variant presentation of maple syrup urine disease. (Q39709016) (← links)
- Branched chain α-ketoacid dehydrogenase kinase 111-130, a T cell epitope that induces both autoimmune myocarditis and hepatitis in A/J mice (Q40166418) (← links)
- Biochemical basis of type IB (E1beta ) mutations in maple syrup urine disease. A prevalent allele in patients from the Druze kindred in Israel (Q40793105) (← links)
- Expression of E1 component of human branched-chain alpha-keto acid dehydrogenase complex in Escherichia coli by cotransformation with chaperonins GroEL and GroES. (Q40855004) (← links)
- Determination of pre-steady-state rate constants on the Escherichia coli pyruvate dehydrogenase complex reveals that loop movement controls the rate-limiting step (Q41141892) (← links)
- Site-directed mutagenesis of a loop at the active site of E1 (alpha2beta2) of the pyruvate dehydrogenase complex. A possible common sequence motif (Q42695311) (← links)
- Carrier detection and rapid newborn diagnostic test for the common Y393N maple syrup urine disease allele by PCR-RFLP: culturally permissible testing in the Mennonite community (Q43696625) (← links)
- Natural osmolyte trimethylamine N-oxide corrects assembly defects of mutant branched-chain alpha-ketoacid decarboxylase in maple syrup urine disease (Q43708605) (← links)
- Identification of two novel BCKDHA mutations in a Chinese patient with maple syrup urine disease (Q43976180) (← links)
- Identification of the E2-binding residues in the N-terminal domain of E1 of a prokaryotic pyruvate dehydrogenase complex (Q44048958) (← links)
- Maple syrup urine disease: mutation analysis in Turkish patients (Q44064412) (← links)
- Insulin increases branched-chain alpha-ketoacid dehydrogenase kinase expression in Clone 9 rat cells (Q44129346) (← links)
- Encapsulation of an 86-kDa assembly intermediate inside the cavities of GroEL and its single-ring variant SR1 by GroES. (Q44216655) (← links)
- Prediction of the binding site on E1 in the assembly of the pyruvate dehydrogenase multienzyme complex of Bacillus stearothermophilus (Q44673411) (← links)
- Structural and biochemical basis for novel mutations in homozygous Israeli maple syrup urine disease patients: a proposed mechanism for the thiamin-responsive phenotype. (Q44741396) (← links)