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198994

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Cláudia Funchal et al.
Journal of the neurological sciences, 217(1), 17-24 (2003-12-17)
In this study we investigated the effects of alpha-ketoisovaleric (KIV) and alpha-keto-beta-methylvaleric acids (KMV), metabolites accumulating in the inherited neurometabolic disorder maple syrup urine disease (MSUD), on the in vitro incorporation of 32P into intermediate filament (IF) proteins from cerebral...
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Susanne Textor et al.
Plant physiology, 144(1), 60-71 (2007-03-21)
Chain elongated, methionine (Met)-derived glucosinolates are a major class of secondary metabolites in Arabidopsis (Arabidopsis thaliana). The key enzymatic step in determining the length of the chain is the condensation of acetyl-coenzyme A with a series of omega-methylthio-2-oxoalkanoic acids, catalyzed...
Nayden Koon et al.
Proceedings of the National Academy of Sciences of the United States of America, 101(22), 8295-8300 (2004-05-26)
The leucine biosynthetic pathway is essential for the growth of Mycobacterium tuberculosis and is a potential target for the design of new anti-tuberculosis drugs. The crystal structure of alpha-isopropylmalate synthase, which catalyzes the first committed step in this pathway, has...
Frances H A Huisman et al.
Biochemistry, 51(11), 2289-2297 (2012-02-23)
α-Isopropylmalate synthase (α-IPMS) catalyzes the metal-dependent aldol reaction between α-ketoisovalerate (α-KIV) and acetyl-coenzyme A (AcCoA) to give α-isopropylmalate (α-IPM). This reaction is the first committed step in the biosynthesis of leucine in bacteria. α-IPMS is homodimeric, with monomers consisting of...
Chris H P van den Akker et al.
Pediatric research, 70(6), 566-571 (2011-08-23)
Human fetal metabolism is largely unexplored. Understanding how a healthy fetus achieves its fast growth rates could eventually play a pivotal role in improving future nutritional strategies for premature infants. To quantify specific fetal amino acid kinetics, eight healthy pregnant...
Vilson de Castro Vasques et al.
Pharmacology, biochemistry, and behavior, 77(1), 183-190 (2004-01-16)
Learning disability is a common feature of patients affected by maple syrup urine disease (MSUD). However, the pathomechanisms underlying learning deficit in this disorder are poorly known. In the present study, we investigated the effect of acute administration of the...
P H Bisschop et al.
The Journal of clinical endocrinology and metabolism, 88(8), 3801-3805 (2003-08-14)
Because insulin is an important regulator of protein metabolism, we hypothesized that physiological modulation of insulin secretion, by means of extreme variations in dietary carbohydrate content, affects postabsorptive protein metabolism. Therefore, we studied the effects of three isocaloric diets with...
Benjamin F Miller et al.
The Journal of physiology, 567(Pt 3), 1021-1033 (2005-07-09)
We hypothesized that an acute bout of strenuous, non-damaging exercise would increase rates of protein synthesis of collagen in tendon and skeletal muscle but these would be less than those of muscle myofibrillar and sarcoplasmic proteins. Two groups (n =...
André Wajner et al.
Metabolic brain disease, 22(1), 77-88 (2007-02-14)
The objective of the present study was to investigate the in vitro effects of the branched-chain alpha-keto acids accumulating in maple syrup urine disease, namely L-2-ketoisocaproic acid, L-2-keto-3-methylvaleric acid and L-2-ketoisovaleric acid on Na(+), K(+)-ATPase activity in synaptic plasma membranes...
Cláudia Funchal et al.
Metabolic brain disease, 20(3), 205-217 (2005-09-17)
In this study we investigate the effects of the branched-chain keto acids (BCKA) alpha-ketoisocaproic (KIC), alpha-ketoisovaleric (KIV), and alpha-keto-beta-methylvaleric (KMV) acids, metabolites accumulating in maple syrup urine disease (MSUD), on the in vitro phosphorylation of glial fibrillary acidic protein (GFAP)...
Andreas Schedlbauer et al.
Journal of biomolecular NMR, 42(2), 111-127 (2008-09-03)
Direct methods in NMR based structure determination start from an unassigned ensemble of unconnected gaseous hydrogen atoms. Under favorable conditions they can produce low resolution structures of proteins. Usually a prohibitively large number of NOEs is required, to solve a...
Joachim Schuster et al.
Plant molecular biology, 57(2), 241-254 (2005-04-12)
Plants are capable to de novo synthesize the essential amino acids leucine, isoleucine and valine. Studies in recent years, however, also revealed that plants have the potential to degrade leucine or may be all of the branched-chain amino acids. One...
Oskar Erikson et al.
Nature biotechnology, 22(4), 455-458 (2004-04-17)
Selectable markers enable transgenic plants or cells to be identified after transformation. They can be divided into positive and negative markers conferring a selective advantage or disadvantage, respectively. We present a marker gene, dao1, encoding D-amino acid oxidase (DAAO, EC...
Felix S Krause et al.
Applied and environmental microbiology, 76(24), 8053-8061 (2010-10-12)
2-Ketoisovalerate is used as a therapeutic agent, and a 2-ketoisovalerate-producing organism may serve as a platform for products deriving from this 2-keto acid. We engineered the wild type of Corynebacterium glutamicum for the growth-decoupled production of 2-ketoisovalerate from glucose by...
Taro Murakami et al.
Journal of nutritional science and vitaminology, 51(1), 48-50 (2005-05-27)
Branched-chain alpha-keto acid dehydrogenase kinase (BDK) phosphorylates and inactivates the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), which is the rate-limiting enzyme in the branched-chain amino acid catabolism. BDK has been believed to be bound to the BCKDC. However, recent our...
Angela M Sgaravatti et al.
Biochimica et biophysica acta, 1639(3), 232-238 (2003-11-26)
Neurological dysfunction is a common finding in patients with maple syrup urine disease (MSUD). However, the mechanisms underlying the neuropathology of brain damage in this disorder are poorly known. In the present study, we investigated the effect of the in...
Cláudia Funchal et al.
Cellular and molecular neurobiology, 26(1), 67-79 (2006-04-25)
Accumulation of the branched-chain alpha-keto acids (BCKA), alpha-ketoisocaproic acid (KIC), alpha-keto-beta-methylvaleric acid (KMV), and alpha-ketoisovaleric acid (KIV) and their respective branched-chain alpha-amino acids (BCAA) in tissues and biological fluids is the biochemical hallmark of patients affected by the neurometabolic disorder...
Marta de la Plaza et al.
Journal of molecular microbiology and biotechnology, 17(2), 96-100 (2008-11-27)
The alpha-ketoisovalerate decarboxylase (Kivd) is a unique lactococcal key enzyme in the decarboxylation of branched-chain alpha-keto acids derived from branched-chain amino acids transamination into aldehydes. These products are important aroma compounds produced during cheese ripening. In this study, the Kivd...
H C Beck et al.
Journal of applied microbiology, 96(5), 1185-1193 (2004-04-14)
Staphylococcus xylosus is an important starter culture in the production of flavours from the branched-chain amino acids leucine, valine and isoleucine in fermented meat products. The sensorially most important flavour compounds are the branched-chain aldehydes and acids derived from the...
Shota Atsumi et al.
Applied and environmental microbiology, 75(19), 6306-6311 (2009-08-18)
A pathway toward isobutanol production previously constructed in Escherichia coli involves 2-ketoacid decarboxylase (Kdc) from Lactococcus lactis that decarboxylates 2-ketoisovalerate (KIV) to isobutyraldehyde. Here, we showed that a strain lacking Kdc is still capable of producing isobutanol. We found that...
Shanshan Li et al.
Applied microbiology and biotechnology, 91(3), 577-589 (2011-05-03)
In the present work, Bacillus subtilis was engineered as the cell factory for isobutanol production due to its high tolerance to isobutanol. Initially, an efficient heterologous Ehrlich pathway controlled by the promoter P(43) was introduced into B. subtilis for the...
An evaluation of the substrate specificity and asymmetric synthesis potential of the cloned L-lactate dehydrogenase from Bacillus stearothermophilus.
Canadian Journal of Chemistry, 67(6), 1065-1070 (1989)
Jordan M Wilkins et al.
Aging, 12(14), 15134-15156 (2020-07-09)
Multiple sclerosis (MS) is a central nervous system inflammatory demyelinating disease and the most common cause of non-traumatic disability in young adults. Despite progress in the treatment of the active relapsing disease, therapeutic options targeting irreversible progressive decline remain limited....
Jan Marienhagen et al.
Journal of bacteriology, 187(22), 7639-7646 (2005-11-04)
Twenty putative aminotransferase (AT) proteins of Corynebacterium glutamicum, or rather pyridoxal-5'-phosphate (PLP)-dependent enzymes, were isolated and assayed among others with L-glutamate, L-aspartate, and L-alanine as amino donors and a number of 2-oxo-acids as amino acceptors. One outstanding AT identified is...
Isabel Ayala et al.
Journal of biomolecular NMR, 43(2), 111-119 (2008-12-31)
A strategy for the introduction of ((1)H,(13)C-methyl)-alanine into perdeuterated proteins is described. Specific protonation of alanine methyl groups to a level of 95% can be achieved by overexpressing proteins in M9/D(2)O based bacterial growth medium supplemented with 800 mg/l of...
Cláudia Funchal et al.
Glia, 48(3), 230-240 (2004-09-25)
Severe neurological symptoms, cerebral edema, and atrophy are common features of the inherited metabolic disorder maple syrup urine disease (MSUD). However, the pathomechanisms involved in the neuropathology of this disease are not well established. In this study, we investigated the...
Jens Buchholz et al.
Applied and environmental microbiology, 79(18), 5566-5575 (2013-07-10)
Exchange of the native Corynebacterium glutamicum promoter of the aceE gene, encoding the E1p subunit of the pyruvate dehydrogenase complex (PDHC), with mutated dapA promoter variants led to a series of C. glutamicum strains with gradually reduced growth rates and...
Raquel Bridi et al.
Metabolic brain disease, 20(2), 155-167 (2005-06-09)
Maple syrup urine disease (MSUD) is an inherited neurometabolic disorder caused by deficiency of branched-chain alpha-keto acid dehydrogenase complex activity which leads to tissue accumulation of the branched-chain alpha-keto acids (BCKAs) alpha-ketoisocaproic acid (KIC), alpha-ketoisovaleric acid (KIV) and alpha-keto-beta-methylvaleric acid...
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