Results 81 to 90 of about 4,253 (187)

Lipoyltransferase 1 Gene Defect Resulting in Fatal Lactic Acidosis in Two Siblings

Case Reports in Obstetrics and Gynecology, 2016
A term male neonate developed severe intractable lactic acidosis on day of life 1 and died the same day at our institution. The family previously lost another term, female newborn on day of life 1 from suspected sepsis at an outside hospital.
Véronique Taché, Liga Bivina, Sophie White, Jeffrey Gregg, Joshua Deignan, Simeon A. Boyadjievd, Francis R. Poulain   +6 more
doaj   +1 more source

Biallelic Mutations in LIPT2 Cause a Mitochondrial Lipoylation Defect Associated with Severe Neonatal Encephalopathy

, 2017
Item does not contain fulltextLipoate serves as a cofactor for the glycine cleavage system (GCS) and four 2-oxoacid dehydrogenases functioning in energy metabolism (alpha-oxoglutarate dehydrogenase [alpha-KGDHc] and pyruvate dehydrogenase [PDHc]), or ...
Laroche, Cécile, Chadefaux-Vekemans, Bernadette, Nicolas Goudin, Lebigot, E., Patrick Nitschke, Stefan Kölker, Ottolenghi, Chris, de Lonlay, P., Delahodde, Agnès, Rotig, Agnes, Pontoizeau, Clément, Nitschke, P., Goudin, N., Tobias B. Haack, Busiah, Kanetee, Haack, T.B., Audrey Boutron, Marquardt, Iris, Lebigot, Elise, Johannes A. Mayr, Delahodde, A., Eisermann, M., Prokisch, H., Boutron, Audrey, Chris Ottolenghi, Bole-Feysot, C., Chretien, D., Hamel, Yamina, Agnès Delahodde, Mayr, J.A., Nathalie Boddaert, Rodenburg, R.J., Yamina Hamel, René G. Feichtinger, Lonlay, Pascale,, Bole-Feysot, Christine, Cécile Laroche, Elise Lebigot, Madrange, M., Grisel, C., Rodenburg, R.J.T., Christine Bole-Feysot, Lonlay, P. de, Meitinger, T., Clément Pontoizeau, Marquardt, I., Coraline Grisel, Habarou, Florence, Laroche, C., Kölker, Stefan, Nemazanyy, I., Kolker, S., Nemazanyy, Ivan, Florence Habarou, Iris Marquardt, Mayr, Johannes A., Monika Eisermann, Eisermann, Monika, Agnes Rotig, Marine Madrange, Pascale de Lonlay, Tim M. Strom, Chrétien, Dominique, Boddaert, Nathalie, G. Christoph Korenke, Boddaert, N., Feichtinger, René G., Strom, T.M., Pontoizeau, C., Kanetee Busiah, Boutron, A., Busiah, K., Korenke, G.C., Haack, Tobias B., Barouki, Robert, Prokisch, Holger, Feichtinger, R.G., Goudin, Nicolas, Meitinger, Thomas, Dominique Chrétien, Richard J. Rodenburg, Strom, Tim M., Barouki, R., Lonlay, Pascale, De, Ottolenghi, C., Rotig, A., Ivan Nemazanyy, Bernadette Chadefaux-Vekemans, Thomas Meitinger, Korenke, G. Christoph, Madrange, Marine, Grisel, Coraline, Rodenburg, Richard J., Chadefaux-Vekemans, B., Nitschke, Patrick, Robert Barouki, Habarou, F., Hamel, Y., Holger Prokisch   +98 more
core   +1 more source

Xenobiotic Incorporation into Pyruvate Dehydrogenase Complex Can Occur Via the Exogenous Lipoylation Pathway

Lipoylated enzymes such as the E2 component of pyruvate dehydrogenase complex (PDC-E2) are targets for autoreactive immune responses in primary biliary cirrhosis, with lipoic acid itself forming a component of the dominant auto-epitopes.
Gray J, Walden HR, Yeaman SJ, Kirby JA, Jones DE, Palmer JM   +5 more
core   +5 more sources

ATP7A and LIAS promote lung adenocarcinoma progression through regulation of cuproptosis

Cancer Cell International
Background Cuproptosis has been implicated in lung adenocarcinoma (LUAD), but whether it contributes to the biological functions of ATPase copper transporting alpha (ATP7A) and lipoic acid synthetase (LIAS) remains unclear.
Wen-Zhou Liu, Fei-Hai Liang, Jian Feng, Yuan Chen, Cheng Cao, You-Rong Chen, Jin-Feng Ge   +6 more
doaj   +1 more source

Staphylococcus aureus exploits lipoic acid salvage to combat host oxidative stress

Cell Reports
Summary: Phagocytic leukocytes employ reactive oxygen species to defend against pathogenic microorganisms. The bacterial pathogen Staphylococcus aureus adapts to oxidative stress by producing antioxidant enzymes and small molecules to protect proteins ...
Iván C. Acosta, Andrew Albers, Liwei Fang, Gustavo Serrato, Wei Ping Teoh, David G. Glanville, Francis Alonzo, III   +6 more
doaj   +1 more source

Xenobiotic incorporation into pyruvate dehydrogenase complex can occur via the exogenous lipoylation pathway

, 2008
Lipoylated enzymes such as the E2 component of pyruvate dehydrogenase complex (PDC-E2) are targets for autoreactive immune responses in primary biliary cirrhosis, with lipoic acid itself forming a component of the dominant auto-epitopes.
Jones, David E., Palmer, Jeremy, Kirby, John, Yeaman, Stephen, Gray, Joe, Walden, Hannah   +5 more
core   +1 more source

The Potential Mechanism of Cuproptosis in Hemocytes of the Pacific Oyster Crassostrea gigas upon Elesclomol Treatment

Cells
Cuproptosis is a novel cell death dependent on mitochondrial respiration and regulated by copper. While the study of it is mainly focused on tumor therapy, in the present study, two key cuproptosis-related genes, ferredoxin (FDX1) and dihydrolipoamide S ...
Yuxin Zhang, Jiejie Sun, Shurong Li, Lingling Wang, Linsheng Song   +4 more
doaj   +1 more source

Lipoyl Dehydrogenase

Journal of Biological Chemistry, 1964
C.J. Lusty, Thomas P. Singer
openaire   +1 more source

Analysis of lipoylation of Lat1p, Kgd2p and Gcv3p.

, 2014
(A) Regions I–III (framed in figure 3) from the 2D gels of wild type (WT) and Tet-Sat4 are shown. Arrowheads indicate spots that are discussed in the text. Spots marked with open arrows represent the respective lipoylated protein form.
Udo Krause (613750), Gerhard Rödel (326147), Cornelia Czupalla (613749), Bernard Hoflack (164600), Uta Gey (613748)   +4 more
core   +1 more source

Sirtuin-regulated lipoylation is an evolutionarily conserved mediator of metabolic health and disease

, 2018
Lipoylation is a rare, but highly conserved lysine posttranslational modification. To date, it is known to occur on only four multimeric metabolic enzymes in mammals, yet these proteins are staples in the core metabolic landscape.
Rowland, Elizabeth
core