A Deep Clinical and Biochemical Characterization of a Patient With Combined Malonic and Methylmalonic Aciduria (CMAMMA). [PDF]
JIMD RepABSTRACT Combined malonic and methylmalonic aciduria (CMAMMA) is an inborn error of metabolism caused by a deficiency in mitochondrial malonyl‐CoA synthetase, the enzyme responsible for activating malonic acid (MA) to malonyl‐CoA, a precursor of lipoic acid.
Gragnaniello V +9 more
europepmc +2 more sources
A unique lipoylation system in the Archaea [PDF]
The FEBS Journal, 2009 Members of the 2-oxoacid dehydrogenase multienzyme complex family play a key role in the pathways of central metabolism. Post-translational lipoylation of the dihydrolipoyl acyltransferase component of these complexes is essential for their activity, the lipoyllysine moiety performing the transfer of substrates and intermediates between the different ...
Mareike G, Posner +4 more
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Lipoylation Mechanism of P. Falciparum Mitochondrial Proteins [PDF]
Biophysical Journal, 2015 Lipoate is an essential cofactor for the aerobic metabolism in oxidative decarboxylation reactions of 2-oxoacid complexes. Malaria parasite survival is highly dependent on scavenging this essential cofactor from the human host. Scavenged lipoate is subsequently attached to the α-ketoglutarate dehydrogenase (KDH), the branched chain α-ketoacid ...
Guerra, Alfredo J. +3 more
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Evidence for two protein‐lipoylation activities in Escherichia coli [PDF]
FEBS Letters, 1991 The lipoate acyltransferase subunits of the 2‐oxo acid dehydrogenase complexes are post‐translationally modified with one or more covalently‐bound lipoyl cofactors. Two distinct lipoate‐protein ligase activities, LPL‐A and LPL‐B, have been detected in E. coli by their ability to modify purified lipoyl apo‐domains of the bacterial pyruvate dehydrogenase
Brookfield, Dawn E. +4 more
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Novel insights into the Thaumarchaeota in the deepest oceans: their metabolism and potential adaptation mechanisms [PDF]
, 2020 Background: Marine Group I (MGI) Thaumarchaeota, which play key roles in the global biogeochemical cycling of nitrogen and carbon (ammonia oxidizers), thrive in the aphotic deep sea with massive populations.
Lehtovirta-Morley, Laura +8 more
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Molecular mechanisms of mitochondrial de novo [2Fe-2S] cluster formation and lipoyl biosynthesis [PDF]
, 2023 Iron-sulfur (Fe/S) clusters are small inorganic protein cofactors found in almost all known organisms. They enable various protein functions including electron transfer and catalysis and are integral to numerous essential biological processes like ...
Schulz, Vinzent
core +1 more source
Overexpressing the H-protein of the glycine cleavage system increases biomass yield in glasshouse and field grown transgenic tobacco plants [PDF]
, 2018 Photorespiration is essential for C3 plants, enabling oxygenic photosynthesis through the scavenging of 2‐phosphoglycolate. Previous studies have demonstrated that overexpression of the L‐ and H‐proteins of the photorespiratory glycine cleavage system ...
Brown, Kenny L +5 more
core +2 more sources
Formation of reactive oxygen species by human and bacterial pyruvate and 2- oxoglutarate dehydrogenase multienzyme complexes reconstituted from recombinant components [PDF]
, 2015 Individual recombinant components of pyruvate and 2-oxoglutarate dehydrogenase multienzyme complexes (PDHc, OGDHc) of human and Escherichia coli (E. coli) origin were expressed and purified from E. coli with optimized protocols.
Adam-Vizi +123 more
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Lipoic Acid Metabolism as a Potential Chemotherapeutic Target Against Plasmodium falciparum and Staphylococcus aureus [PDF]
, 2021 Lipoic acid (LA) is an organic compound that plays a key role in cellular metabolism. It participates in a posttranslational modification (PTM) named lipoylation, an event that is highly conserved and that occurs in multimeric metabolic enzymes of very ...
Du, Xiaochen +4 more
core +1 more source
A Lipoylated Metabolic Protein Released by Staphylococcus aureus Suppresses Macrophage Activation [PDF]
Cell Host & Microbe, 2017 The virulence factors of pathogenic microbes often have single functions that permit immune suppression. However, a proportion possess multiple activities and are considered moonlighting proteins. By examining secreted virulence factors of Staphylococcus aureus, we determine that the bacterial lipoic acid synthetase LipA suppresses macrophage ...
James P, Grayczyk +3 more
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