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1 d with ruthenium tetroxide provided the seco ketoacid.
2 tion by one-carbon extension cycles of alpha-ketoacids.
3 yze the decarboxylation of a series of alpha-ketoacids.
4 oxyalkanes followed by carboxylation to beta-ketoacids.
5 mimics of peptide alpha-ketoesters and alpha-ketoacids.
6 liphatic, and aromatic carboxylic acids, and ketoacids.
7 and the degradation of branched-chain alpha-ketoacids.
8 tive amide-bond forming reactions with alpha-ketoacids.
9 cally enriched, side chain unprotected alpha-ketoacids.
10 phatic nitroalkenes, 1,3-diketones, and beta-ketoacids.
11 e Ugi 4-center-3-component reaction of gamma-ketoacids.
12 me family that oxidizes L-2-hydroxy acids to ketoacids.
13 n of aryl alkyl ketones to the corresponding ketoacids.
14 hat oxidizes (S)-alpha-hydroxyacids to alpha-ketoacids.
15 thway resulting in net carboxylation to beta-ketoacids.
16 anobacterial sunscreen, have identified beta-ketoacid 2 as an important intermediate that is produced
19 nhydrolyzable pTyr mimics that contain alpha-ketoacid, alpha-hydroxyacid, and methylenesulfonamide fu
20 queous conditions produces a series of alpha-ketoacid analogues of the reductive citric acid cycle wi
22 and V/K varied substantially when different ketoacid and pyridine nucleotide substrates were used.
23 erium isotope effects were observed for poor ketoacid and pyridine nucleotide substrates, indicating
24 tial to modify E2 subunits of branched chain ketoacid and pyruvate dehydrogenases during lipoate scav
25 est yields were obtained when both the alpha-ketoacid and the N-hydroxylamino acid contained medium-s
26 pproximately 36 h) with a 1:2 ratio of alpha-ketoacids and 2 or 3 gave major yields of the alpha,alph
31 construction of bicyclic beta-lactones from ketoacids and implements the use of commercially availab
32 is shown to contribute to the synthesis of a-ketoacids and important odor-active esters in apple (Mal
34 tes of fatty acid biosynthesis) to release 3-ketoacids and that ShMKS1 subsequently catalyzes the dec
35 terase producing long-chain (mainly C(16)) 3-ketoacids, and another one as a polyketide synthase (PKS
36 ranched-chain amino acids and branched-chain ketoacids, and this buildup has been associated with hea
37 ; 'K', the presence of high urinary or blood ketoacids; and 'A', a high anion gap metabolic acidosis.
38 amino acids (BCAAs) and branched-chain alpha-ketoacids are associated with cardiovascular and metabol
39 carboxylation of aliphatic epoxides to beta-ketoacids as illustrated by the reaction epoxypropane +
41 d-chain amino acid (BCAA) and branched-chain ketoacid (BCKA) ingestion on postprandial muscle protein
42 phenes demonstrated sustained branched chain ketoacid (BCKA) lowering and reduced BDK protein levels,
43 chain amino acids (BCAA) and their cognate a-ketoacids (BCKA) are elevated in an array of cardiometab
44 or supplementation with branched-chain alpha-ketoacids (BCKA), downstream metabolic products of BCAT1
45 omarkers, including BCAAs and branched-chain ketoacids (BCKAs), were lowered in vivo with enhanced ph
47 branched-chain amino acids (BCAAs) and their ketoacids but lower ketoisocaproate (KIC)-to-Leu, ketome
48 then undergoes two consecutive sets of alpha-ketoacid chain elongation reactions to produce alpha-ket
49 that elevated skeletal muscle succinyl CoA:3-ketoacid CoA transferase (SCOT) activity, the rate-limit
50 ed by an increase activity of succinyl-CoA:3-ketoacid-CoA transferase (SCOT) activity, the rate-limit
51 s can use a pathway involving succinyl-CoA:3-ketoacid-CoA transferase and acetoacetyl-CoA synthetase
52 lets possessed high levels of succinyl-CoA:3-ketoacid-CoA transferase, an enzyme that forms acetoacet
54 ouples oxidative decarboxylation of an alpha-ketoacid cofactor to oxidative modification of its subst
55 d also produced relatively more hydroxy- and ketoacid compounds that have implications for the fresh-
56 me A-dependent oxidation of branched-chain 2-ketoacids coupled to the reduction of viologen dyes or f
58 The human mitochondrial branched-chain alpha-ketoacid decarboxylase/dehydrogenase (BCKD) is a heterot
60 ental and computational study here of a beta-ketoacid decarboxylation shows how the distinction betwe
61 ular accumulation of their substrates (alpha-ketoacids), decrease of their products (acyl-CoAs), and
63 nknown, the function of branched-chain alpha-ketoacid dehydrogenase (BCKAD), the rate-limiting enzyme
65 The mammalian mitochondrial branched-chain ketoacid dehydrogenase (BCKD) complex is a multienzyme c
66 ing to the genes of the branched-chain alpha-ketoacid dehydrogenase (BCKD) complex which are affected
67 rough inhibition of the branched-chain-alpha-ketoacid dehydrogenase (BCKD) complex, the rate-limiting
69 previously that the rat branched-chain alpha-ketoacid dehydrogenase (BCKD) kinase is capable of autop
70 or of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) responsible for the rate-l
71 of human mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD), chaperonins GroEL/GroES i
73 Although deficiency of the branched-chain ketoacid dehydrogenase (BCKDC) and associated elevations
76 ations in two subunits of the branched-chain ketoacid dehydrogenase (BCKDH) complex, a key enzyme com
79 decarboxylation by the enzyme branched chain ketoacid dehydrogenase (BCKDH), which is negatively regu
80 cellular BCKAs are cleared by branched-chain ketoacid dehydrogenase (BCKDH), which is sensitive to in
82 d synthesis, are derived from branched-chain ketoacid dehydrogenase (Bkd), a multiprotein complex tha
83 he active site of human branched-chain alpha-ketoacid dehydrogenase (E1b) impede both the decarboxyla
84 iral complementation of branched-chain alpha-ketoacid dehydrogenase activity to identify the gene loc
85 component common to the mitochondrial alpha-ketoacid dehydrogenase and glycine decarboxylase complex
86 he similarity of murine branched chain alpha-ketoacid dehydrogenase and its kinase to the human enzym
87 al similarity of murine branched chain alpha-ketoacid dehydrogenase and its regulation by the complex
90 component of the human branched-chain alpha-ketoacid dehydrogenase complex (BCKDC) has been expresse
93 The purified mammalian branched-chain alpha-ketoacid dehydrogenase complex (BCKDC), which catalyzes
94 ed by the mitochondrial branched-chain alpha-ketoacid dehydrogenase complex (BCKDC), which is negativ
95 d for regulation of the branched chain alpha-ketoacid dehydrogenase complex by kinase expression duri
96 E2 of the mitochondrial branched-chain alpha-ketoacid dehydrogenase complex can cause the disease.
97 xylase component of the human branched-chain ketoacid dehydrogenase complex comprises two E1alpha (45
98 BCKDC belongs to the mitochondrial alpha-ketoacid dehydrogenase complex family, which also includ
99 (E2b) component of the branched-chain alpha-ketoacid dehydrogenase complex forms a cubic scaffold th
100 erentially metabolized by the branched-chain ketoacid dehydrogenase complex, in contrast to valine, w
105 d (LA) is a cofactor for mitochondrial alpha-ketoacid dehydrogenase complexes and is one of the most
106 arate dehydrogenase and branched chain alpha-ketoacid dehydrogenase complexes and that the apicoplast
107 y a single gene and shared between the alpha-ketoacid dehydrogenase complexes and the Gly decarboxyla
108 n enzyme could restore function to the alpha-ketoacid dehydrogenase complexes in a yeast strain defic
109 yltransferase (SucB) are components of alpha-ketoacid dehydrogenase complexes that are central to int
110 ed for catalysis by multiple mitochondrial 2-ketoacid dehydrogenase complexes, including pyruvate deh
111 tioxidant and an essential cofactor in alpha-ketoacid dehydrogenase complexes, which participate in g
115 xpression of a putative branched-chain alpha-ketoacid dehydrogenase E1 beta-subunit-encoding gene (Na
116 he L-pyruvate kinase and islet amyloid chain ketoacid dehydrogenase E1a promoter, but it does not aff
117 n genes that encode the branched-chain alpha-ketoacid dehydrogenase E1alpha (BCKDHA), E1beta (BCKDHB)
118 ed expression and activity of branched-chain ketoacid dehydrogenase enzyme, many muscle amino metabol
120 DC), which belong to the mitochondrial alpha-ketoacid dehydrogenase family, play crucial roles in cel
121 easome proteolytic system and branched-chain ketoacid dehydrogenase in muscle, along with hepatic glu
125 eractors and found that branched-chain alpha-ketoacid dehydrogenase kinase (BCKDK) is an in vivo UBE3
127 egulation of the BCKDC by the branched-chain ketoacid dehydrogenase kinase has also been implicated i
128 mutations in the gene BCKDK (Branched Chain Ketoacid Dehydrogenase Kinase) in consanguineous familie
130 the human mitochondrial branched chain alpha-ketoacid dehydrogenase multienzyme complex (approximatel
131 m genome contains genes encoding three alpha-ketoacid dehydrogenase multienzyme complexes (KADHs) tha
133 uding those encoding putative branched-chain ketoacid dehydrogenase subunits, is highly expressed dur
134 lutarate dehydrogenase, branched-chain alpha-ketoacid dehydrogenase, and the glycine cleavage system.
135 -ketoglutarate dehydrogenase, branched chain-ketoacid dehydrogenase, and the glycine cleavage system.
136 nched chain aminotransferase, branched chain ketoacid dehydrogenase, glutamate dehydrogenase, and glu
141 tional lipoic arms across the multiple alpha-ketoacid dehydrogenases and led to intracellular accumul
142 icient in LIPT1, the enzyme that activates 2-ketoacid dehydrogenases related to the TCA cycle(4,5).
143 LIPT1 covalently conjugates mitochondrial 2-ketoacid dehydrogenases with lipoic acid, facilitating e
144 ted that RNS can generally inhibit all alpha-ketoacid dehydrogenases, which has broad physiological i
147 ochondrial BCKDH complex that decarboxylates ketoacid derivatives of leucine, isoleucine, and valine.
148 ; Km < 100 microM) for the enzyme were the 2-ketoacid derivatives of valine, leucine, isoleucine, and
149 LipDH and mitochondrial branched chain alpha-ketoacid dihydrolipoamide transacylase in these parasite
150 cid is produced through the one-carbon alpha-ketoacid elongation pathway with the involvement of the
151 s of intracellular glutathione, NADPH, alpha-ketoacids, ferredoxin, and thioredoxin indicated that no
153 to an effect specific to KIC rather than to ketoacids generally, and argues against an antioxidant m
154 ard other straight- and branched-chain alpha-ketoacids, greatly expanding the range of substrates pre
155 e in situ generated acyl radicals from alpha-ketoacids have been coupled to a wide variety of electro
156 associated elevations in the BCAAs and their ketoacids have been recognized as the cause of maple syr
160 ng of apple tissue labeled citramalate and a-ketoacids in a manner consistent with the presence of th
164 -bound enolate intermediates formed from the ketoacids in the presence of the peptide coupling reagen
166 riants perform addition of beta-fluoro-alpha-ketoacids (including fluoropyruvate, beta-fluoro-alpha-k
167 cei excretes significant amounts of aromatic ketoacids, including indolepyruvate, a transamination pr
168 acid production have motivated the use of 2-ketoacid intermediates for the production of important c
171 precursors bearing hydroxylamines and alpha-ketoacids (KAs) or potassium acyltrifluoroborates (KATs)
172 lar aldol lactonization of readily available ketoacids leading to the enantioselective synthesis of c
175 s, glycopeptide I, which contained the alpha-ketoacid moiety at the C-terminus, were synthesized and
178 metabolites upstream of branched-chain alpha-ketoacid oxidation, consistent with reduced BCKD activit
179 uriosus contains four distinct cytoplasmic 2-ketoacid oxidoreductases (ORs) which differ in their sub
180 te is often not detected in studies of alpha-ketoacid oxidoreductases because it rapidly decays.
181 eadily converted to C-terminal peptide alpha-ketoacids, poised for chemoselective amide-forming react
183 ne) and dioxygen to generate formate and the ketoacid precursor of methionine, 2-keto-4-methylthiobut
186 fatty acyl-CoA substrates to produce a beta-ketoacid product and initiates the biosynthesis of long
187 7a-d) result from one-pot reactions of alpha-ketoacids, RCOCO(2)H (R = C(6)H(5), CH(3), CH(3)CH(2), t
189 bited when incubated in branched-chain alpha-ketoacids, saturated and unsaturated fatty acids, or 5-a
190 ata are consistent with binding of the alpha-ketoacid substrate by this residue based on the Pseudomo
192 ersion of metabolism to build and break down ketoacids, sugars, amino acids, and ribonucleotides in m
193 e, a pivotal glucose metabolite, is an alpha-ketoacid that reacts with hydrogen peroxide (H(2)O(2)).
195 ver, biology employs transamination of alpha-ketoacids to synthesize amino acids which are then trans
197 Duplications of the bidirectional alpha-ketoacid transporters SLC16A3, SLC16A7, the cystine tran
198 one oxidant, followed by alkylation with a B-ketoacid under mild conditions to provide valuable B-ami
199 oxidant, followed by alkylation with a beta-ketoacid under mild conditions to provide valuable beta-
203 that intracellular pyruvate, or other alpha-ketoacids, whose endogenous concentration is controlled
204 recently developed novel pathways based on 2-ketoacids will be described along with representative ex
205 a polyketide synthase (PKS) condensing the 3-ketoacids with long-chain (mainly C(16)) acyl-CoAs into