Methylcrotonyl-CoA
Albert, Christoph (2009): Die humane 3-Methylcrotonyl-CoA-Carboxylase: Entwicklung eines prokaryontischen Expressionssystems Generierung von Antikörpern zum Nachweis der alpha- und beta-Untereinheit. Dissertation, LMU München: Medizinische Fakultät
... Die 3-Methylcrotonyl-CoA-Carboxylase gehört zu den biotinabhängigen Carboxylasen. Die α-Untereinheit trägt ein kovalent gebundenes Biotin, welches zur Affinitätsreinigung mit einer Avidin-Matrix genutzt ...
116
The molecular basis of human 3 methylcrotonyl CoA carboxylase deficiency
... Isolated biotin-resistant 3-methylcrotonyl-CoA carboxylase (MCC) deficiency is an autosomal recessive disorder of leucine catabolism that appears to be the most frequent organic aciduria detected in tandem ...
11
Lagler, Florian Bartholomaeus (2002): 3-Methylcrotonyl-CoA: Carboxylase Mangel: Molekulare Grundlagen. Dissertation, LMU München: Medizinische Fakultät
... Der isolierte 3-Methylcrotonyl-CoA: Carboxylase (MCC) Mangel ist eine angeborene Störung im Abbau der Aminosäure Leucin. Es sind sowohl bis ins Erwachsenenalter asymptomatische als auch frühe letale ...
50
Stadler, Sonja (2006): Die humane 3-Methylcrotonyl-CoA-Carboxylase: Charakterisierung des Enzyms und des Enzymdefektes. Dissertation, LMU München: Fakultät für Biologie
... 1959 wurde von 3-MCC als einem Enzym berichtet, welches an der Carboxylierung von 3-Methylcrotonyl-CoA in Hühnerleber beteiligt ist (Del Campillo-Campbell et al., 1959). Zwei Jahre später konnten Lynen et ...
153
BETA-METHYLCROTONYL-CoA CARBOXYLASE DEFICIENCY: A NEW METABOLIC ERROR IN LEUCINE DEGRADATION
... missions the serum level of isovaleric acid is in the order of 0.2 to 0.5 mg/ 100 ml and the urinary excretion of 3-hydroxyisovaleric. acid 1 to 7 mg/24 hours[r] ...
12
3-methylcrotonyl-CoA carboxylase deficiency: Clinical, biochemical, enzymatic and molecular studies in 88 individuals
... The phenomenon that mutations may cause a clear bio- chemical phenotype in otherwise asymptomatic individuals is well known from other inborn errors of metabolism implemented in expanded NBS such as isovaleric acidemia ...
24
Molecular, genetic, physiological and biochemical studies of 3 methylcrotonyl CoA carboxylase and biotin carboxyl carrier protein like proteins in Arabidopsis thaliana
... When illumination is withdrawn for a prolonged time, photosynthesis is ceased and the plant is under sugar starvation. Reserves, such as fatty acids and proteins are being remobilized to provide energy for survival. ...
169
Polanetz, Roman H. (2006): Identifizierung und molekulare Charakterisierung mitochondrialer Signalpeptide der 3-Methylcrotonyl-CoA-Carboxylase. Dissertation, LMU München: Medizinische Fakultät
... Hierdurch konnte gezeigt werden, dass die aminoterminalen 20 Aminosäuren ausreichend und notwendig sind, damit MCCβ in das Mitochondrium importiert werden kann. Die Größe des[r] ...
68
Potential Misdiagnosis of 3-Methylcrotonyl-Coenzyme A Carboxylase Deficiency Associated With Absent or Trace Urinary 3-Methylcrotonylglycine
... the likely candidate for catalysis of 3-methylcrotonyl- CoA conjugation with glycine (Fig 1). It remains possible that our patients have an abnormality of acyltransferase activity, located on chromosome 11, ...
8
Deficiency of 3 methylglutaconyl coenzyme A hydratase in two siblings with 3 methylglutaconic aciduria
... We studied two patients with 3-methylglutaconic aciduria in order to determine the molecular defect. A new assay for 3-methylglutaconyl-coenzyme A (CoA) hydratase has been developed in which the substrate, ...
6
Clinical and biochemical characterization of four patients with mutations in ECHS1
... methacrylyl- CoA in other organs, or the residual SCEH activity with crotonyl-CoA and tiglyl-CoA, but not methacrylyl-CoA, is sufficient to maintain flux through the respective catabolic ...
15
Biotin response Organicaciduria: MULTIPLE CARBOXYLASE DEFECTS AND COMPLEMENTATION STUDIES WITH PROPIONICACIDEMIA IN CULTURED FIBROBLASTS
... Fibroblast cultures from two individuals with biotin-responsive organicacidemia were found to have a pleiotropic deficiency of propionyl-CoA carboxylase, b-methylcrotonyl-CoA carboxylase[r] ...
9
Production of 3 hydroxypropionate from biomass
... Biorenewable technology is a developing field of science that researches alternative sources for petroleum products like fuels, plastics, paints, etc. The DoE biomass program has identified the 12 top chemicals that can ...
59
Measurement of δ Aminolevulinic Acid Synthetase Activity in Human Erythroblasts
... A new, specific, and simple method for the determination of d-aminolevulinic acid (ALA) synthetase activity in human bone marrow cells has been developed. ALA synthetase of erythroblasts was partially purified so as to ...
10
Late Diagnosis of Coarctation Despite Prenatal Ultrasound and Postnatal Pulse Oximetry
... The first signs of heart disease in the 83 infants not detected by POS were noted at a median age of 2.2 days (range: 0–41.5 days). Forty-three presented with a systolic murmur. Of these, 21 had weak femoral pulses (Table ...
9
The Effect of Increased Ambient CO2 on Arterial CO2 Tension, CO2 Content and pH in Rainbow Trout
... The effect of exposure of rainbow trout to high and low levels of environmental water CO2 tension on blood COa and pH was studied.. An increase in environmental COa caused a rise in the [r] ...
8
HMG-CoA reductase inhibitor lovastatin upregulates plasminogen activator production through RhoA-signaling in peritoneal cell line Met5A
... Hydroxymethylglutaryl-CoA (HMG-CoA) reductase is the rate-limiting enzyme in the conversion of HMG-CoA to mevalonate, an intermediate in the de novo synthesis of cholesterol [5–7]. Apart from the ...
5
Identification of 3 methylglutarylcarnitine A new diagnostic metabolite of 3 hydroxy 3 methylglutaryl coenzyme A lyase deficiency
... Deficiency of 3-hydroxy-3-methylglutaryl-coenzyme A (CoA) lyase affects the metabolism of leucine as well as ketogenesis. This disorder is one of an increasing list of inborn errors of metabolism that presents ...
5
CHANGES IN OIL AND FATTY ACID COMPOSITION OF DEVELOPING SEEDS OF TARAMIRA ( ERUCA SATIVA MILL.) AS INFLUENCED BY SULPHUR AND NITROGEN NUTRITION
... Interactive effect of sulphur and nitrogen on lipid accumulation, acetyl-CoA concentration and acetyl-CoA carboxylase activity in developing seeds of oilseed crops ( Brassica campestris [r] ...
8
Efficient production of glycyrrhetinic acid in metabolically engineered Saccharomyces cerevisiae via an integrated strategy
... pyrophosphate; GPP: geranyl diphosphate; FPP: farnesyl diphosphate; GGPP: geranylgeranyl pyrophosphate; ERG10: acetyl-CoA C-acetyltransferase; ERG13: hydroxymethylglutaryl-CoA synthase;[r] ...
15