Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease
about
Solution structure and dynamics of the lipoic acid-bearing domain of human mitochondrial branched-chain alpha-keto acid dehydrogenase complexCross-talk between thiamin diphosphate binding and phosphorylation loop conformation in human branched-chain alpha-keto acid decarboxylase/dehydrogenaseBinding of pyruvate dehydrogenase to the core of the human pyruvate dehydrogenase complexRoles 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 siteMaple syrup urine disease: identification and carrier-frequency determination of a novel founder mutation in the Ashkenazi Jewish population.Milk--A Nutrient System of Mammalian Evolution Promoting mTORC1-Dependent TranslationThe pathogenic role of persistent milk signaling in mTORC1- and milk-microRNA-driven type 2 diabetes mellitusStructures of the human pyruvate dehydrogenase complex cores: a highly conserved catalytic center with flexible N-terminal domains.Structural basis for flip-flop action of thiamin pyrophosphate-dependent enzymes revealed by human pyruvate dehydrogenaseThe two active sites in human branched-chain alpha-keto acid dehydrogenase operate independently without an obligatory alternating-site mechanismA dual conformation of the post-decarboxylation intermediate is associated with distinct enzyme states in mycobacterial KGD (α-ketoglutarate decarboxylase)Gene preference in maple syrup urine disease.BCKDH: the missing link in apicomplexan mitochondrial metabolism is required for full virulence of Toxoplasma gondii and Plasmodium bergheiNovel isoenzyme of 2-oxoglutarate dehydrogenase is identified in brain, but not in heartHuman microRNA (miR29b) expression controls the amount of branched chain alpha-ketoacid dehydrogenase complex in a cell.Structural basis for activation of the thiamin diphosphate-dependent enzyme oxalyl-CoA decarboxylase by adenosine diphosphate.Functional anthology of intrinsic disorder. 3. Ligands, post-translational modifications, and diseases associated with intrinsically disordered proteins.Roles of active site and novel K+ ion-binding site residues in human mitochondrial branched-chain alpha-ketoacid decarboxylase/dehydrogenase.Serum Metabolomic Profiling of Piglets Infected with Virulent Classical Swine Fever Virus.Probing the mechanism of inactivation of human pyruvate dehydrogenase by phosphorylation of three sites.Maple syrup urine disease in Cypriot families: identification of three novel mutations and biochemical characterization of the p.Thr211Met mutation in the E1alpha subunit.Molecular architecture and mechanism of an icosahedral pyruvate dehydrogenase complex: a multifunctional catalytic machinePhenylbutyrate therapy for maple syrup urine disease.Asp295 stabilizes the active-site loop structure of pyruvate dehydrogenase, facilitating phosphorylation of ser292 by pyruvate dehydrogenase-kinase.A structural perspective on enzymes activated by monovalent cations.Milk: an epigenetic amplifier of FTO-mediated transcription? Implications for Western diseases.Role of Na+ and K+ in enzyme function.Nuclear magnetic resonance approaches in the study of 2-oxo acid dehydrogenase multienzyme complexes--a literature review.Thermodynamic analysis of the binding of component enzymes in the assembly of the pyruvate dehydrogenase multienzyme complex of Bacillus stearothermophilus.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.Molecular Mechanisms of Enzyme Activation by Monovalent Cations.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.Branched chain α-ketoacid dehydrogenase kinase 111-130, a T cell epitope that induces both autoimmune myocarditis and hepatitis in A/J mice.Biochemical basis of type IB (E1beta ) mutations in maple syrup urine disease. A prevalent allele in patients from the Druze kindred in Israel.Determination of pre-steady-state rate constants on the Escherichia coli pyruvate dehydrogenase complex reveals that loop movement controls the rate-limiting step.Site-directed mutagenesis of a loop at the active site of E1 (alpha2beta2) of the pyruvate dehydrogenase complex. A possible common sequence motif.Natural osmolyte trimethylamine N-oxide corrects assembly defects of mutant branched-chain alpha-ketoacid decarboxylase in maple syrup urine disease.Identification of the E2-binding residues in the N-terminal domain of E1 of a prokaryotic pyruvate dehydrogenase complex.Insulin increases branched-chain alpha-ketoacid dehydrogenase kinase expression in Clone 9 rat cells.Encapsulation of an 86-kDa assembly intermediate inside the cavities of GroEL and its single-ring variant SR1 by GroES.
P2860
Q24292283-4ACE6D55-1372-4557-8C3B-79B492BB07C0Q24294487-B4BD6050-B367-4FDD-90E1-9778E63164A9Q24307378-D1B24D4C-F887-4AC1-B5C7-B33ECAEABB28Q24313928-0A283EEC-9851-4D3E-A529-E8D36DBCBD82Q24533417-EC09D6E7-B0DA-4D6C-8F33-B06F63D37485Q26801523-74818D0D-F377-4B61-84A2-A6A177BC6E17Q27027262-6AB4CC1B-B277-4A45-AE2D-31E92D61BE40Q27303634-303BF9A6-63EA-4CD1-BC1E-6B2663AA828BQ27640758-54143909-5A9C-4A9E-A363-520C344DCC1AQ27643936-74BFB59A-923D-42D5-83A8-AEC82F646B28Q27687388-A2B6173D-C053-467F-8657-8C70F59BE2A8Q28345008-47754D47-BA8E-4313-8B9A-943AEC9A7EAEQ28540877-4CA79F72-AA49-4FF7-8235-08FDA53815EAQ29396800-381FEDFD-5295-4EC3-83A0-F89E4803735DQ30351534-ABA331C7-F34E-4D48-8DFC-A9F06FBC386EQ30351586-5A0BB629-0B5C-422A-B3A2-A634F0E1F27BQ30360723-74FAFBC4-EBB8-425D-9376-909C9E9FB37EQ31538248-7EDB9E9C-CAA7-4102-85D4-3693D5E4B03DQ33606965-A4C20699-3F01-438F-89D1-A38ECD03AE70Q33926252-9F139D4A-28BC-4ED0-8577-B0082AE8356DQ34190871-8131D8B8-471E-4C1E-A1B9-35A0335EC7AEQ34206769-EE262253-CEF4-41BD-A7A2-F1017B6A8B50Q34505609-9BA69AA8-26D6-4BEF-A223-6A3678D007F8Q34557385-44F41F37-4066-4C95-99BF-653EB24475E7Q36305162-3E81727D-8634-4911-828A-38295FF4CA41Q36393233-E06BEE1D-4D7F-4DF9-8E0E-64DF295F6951Q36610450-660EB120-D3B6-4CFD-BB05-AC9603FBC64CQ38146650-8C63EB87-1CD9-4BF8-9029-C869D761B80AQ38269524-BD9EA48C-2798-4914-A321-1DD1C4B7D714Q38296259-65125C4A-564C-4FBB-B348-91D512BCA18CQ38909804-52E8F88E-7EEF-4B5B-B5E3-E5CE1B05E363Q39709016-56F9C4FA-9D8C-46A7-873A-D27669F8AC00Q40166418-84D9CD00-74CA-43E8-9F9A-2A45B87B20F5Q40793105-F3C6E114-0402-4B23-9259-292947F65A0EQ41141892-5466458E-611A-48BB-8CD2-764FB57BB6B9Q42695311-7C6F075F-3156-499A-831D-4FB3005D3C6FQ43708605-4C9F0A69-FAC7-4CF4-A84E-FC808883E44DQ44048958-835AE7E0-0DE1-410A-A0FC-8DFC20538446Q44129346-4DA4FA9A-5AF0-4B8E-BE45-AE11D7DF40FFQ44216655-CA07E272-477C-442D-A75F-E1EBC55BB005
P2860
Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease
description
2000 nî lūn-bûn
@nan
2000 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2000 թվականի մարտին հրատարակված գիտական հոդված
@hy
2000年の論文
@ja
2000年論文
@yue
2000年論文
@zh-hant
2000年論文
@zh-hk
2000年論文
@zh-mo
2000年論文
@zh-tw
2000年论文
@wuu
name
Crystal structure of human bra ...... y in maple syrup urine disease
@ast
Crystal structure of human bra ...... y in maple syrup urine disease
@en
Crystal structure of human bra ...... y in maple syrup urine disease
@en-gb
Crystal structure of human bra ...... y in maple syrup urine disease
@nl
type
label
Crystal structure of human bra ...... y in maple syrup urine disease
@ast
Crystal structure of human bra ...... y in maple syrup urine disease
@en
Crystal structure of human bra ...... y in maple syrup urine disease
@en-gb
Crystal structure of human bra ...... y in maple syrup urine disease
@nl
prefLabel
Crystal structure of human bra ...... y in maple syrup urine disease
@ast
Crystal structure of human bra ...... y in maple syrup urine disease
@en
Crystal structure of human bra ...... y in maple syrup urine disease
@en-gb
Crystal structure of human bra ...... y in maple syrup urine disease
@nl
P2093
P1433
P1476
Crystal structure of human bra ...... y in maple syrup urine disease
@en
P2093
P304
P356
10.1016/S0969-2126(00)00105-2
P407
P577
2000-03-15T00:00:00Z