Results 161 to 170 of about 3,788 (176)

Defect in the lipoyl-bearing protein X subunit of the pyruvate dehydrogenase complex in two patients with encephalomyelopathy

The Journal of Pediatrics, 1993
Among the many metabolic encephalomyelopathies caused by deficiencies in the pyruvate dehydrogenase complex (PDHC), nearly all involve its E1 subunit. We describe two new familial cases of PDHC deficiency with encephalomyelopathy, chronic lactic acidemia, and a normal E1 subunit of PDHC but deficiency in another component. Activity of PDHC was measured
C, Marsac, D, Stansbie, G, Bonne, J, Cousin, P, Jehenson, C, Benelli, J P, Leroux, G, Lindsay   +7 more
openaire   +2 more sources

Oligocopper‐Loaded Lipoic Acid Nanoparticles Promote Mitochondrial Protein Lipoylation for Augmented Cuproptosis Therapy

Small
Abstract Cuproptosis is a modality of mitochondrial cell death driven by the binding of excess copper ions to the lipoic acid residues of dihydrolipoamide S‐acetyltransferase (DLAT) protein. However, copper levels are tightly regulated within cells. Herein, an oligocopper‐loaded lipoic acid nanoparticle (Cu@TcNAs)
Ying Chen
exaly   +3 more sources

Phylogenetic and immunological definition of four lipoylated proteins from Novosphingobium aromaticivorans, implications for primary biliary cirrhosis

Journal of Autoimmunity, 2005
Novosphingobium aromaticivorans, a unique ubiquitous bacterium that metabolizes xenobiotics and activates environmental estrogens, has been suggested as a pathogenic factor in the development of primary biliary cirrhosis (PBC). To define the molecular basis of PBC sera reactivity, we investigated the characteristic of the bacterial antigens involved ...
K. A. Padgett, C. Selmi, T. P. Kenny, P. S. Leung, D. L. Balkwill, A. A. Ansari, R. L. Coppel, M. E. Gershwin   +7 more
openaire   +3 more sources

Lipoyl dehydrogenase catalyzes reduction of nitrated DNA and protein adducts using dihydrolipoic acid or ubiquinol as the cofactor

Chemico-Biological Interactions, 2002
Inflamed tissues generate reactive nitrogen oxide species (RNO(x)), such as peroxynitrite (ONOO-)and nitryl chloride (NO2Cl), which lead to formation of nitrated DNA and protein adducts, including 8-nitroguanine (8NG), 8-nitroxanthine (8NX), and 3-nitrotyrosine (3NT).
Hauh-Jyun Candy, Chen, Yuan-Mao, Chen, Chia-Ming, Chang   +2 more
openaire   +2 more sources

Bacterial lipoate protein ligases rescue lipoylation and respiration deficiency in mammals

Summary Human lipoylation pathway deficiencies caused by LIPT2/LIAS/LIPT1 gene mutations lead to inherited metabolic disorders characterized by severe defects of mitochondrial energy and amino acids metabolism. Patients with such mutations suffer from hyperlactic acidemia, encephalopathy, hypotonia and early death. So
Zhijuan Hu, Junru Yu, Ziwei Liu, Min Jiang, An-Ping Zeng   +4 more
openaire   +1 more source

Chemical Tagging of Protein Lipoylation

Angewandte Chemie - International Edition, 2021
Qi Tang, Yilan Guo, Xing Chen
exaly  

Faculty Opinions recommendation of Copper induces cell death by targeting lipoylated TCA cycle proteins.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature, 2022
Yi Lu, Weijie Guo
openaire   +2 more sources

Assay for protein lipoylation reaction.

Methods in enzymology, 1995
K, Fujiwara, K, Okamura-Ikeda, Y, Motokawa   +2 more
openaire   +1 more source

Activation and Transfer of Lipoic Acid in Protein Lipoylation in Mammals

2003
Kazuko Fujiwara, Kazuko Okamura-Ikeda, Yutaro Motokawa   +2 more
openaire   +1 more source

Two redundant octanoyltransferases and one obligatory lipoyl synthase provide protein‐lipoylation autonomy to plastids of Arabidopsis

Plant Biology, 2014
C Hoffmann
exaly