Results 61 to 70 of about 4,273 (194)

Clinical and biochemical characterization of four patients with mutations in ECHS1 [PDF]

, 2015
Short-chain enoyl-CoA hydratase (SCEH, encoded by ECHS1) catalyzes hydration of 2-trans-enoyl-CoAs to 3(S)-hydroxy-acyl-CoAs. SCEH has a broad substrate specificity and is believed to play an important role in mitochondrial fatty acid oxidation and in ...
Alberto Burlina, Curtis R. Coughlin, Frédéric M. Vaz, Hans R. Waterham, Jirair K. Bedoyan, Johan L. K. Van Hove, Jos P. N. Ruiter, Kaitlyn Bloom, Katherine Gowan, Kathryn Chatfield, Marisa W. Friederich, Megan K. Dishop, Michael J. Bennett, Orly Elpeleg, Renata C. Gallagher, Ronald J. A. Wanders, Sacha Ferdinandusse   +16 more
core   +1 more source

Asymmetric Cu─N─Ru Bridgedsite Nanozyme‐Loaded Injectable Thermogel Boosts Cuproptosis‐Like Death for Multidrug‐Resistant Urinary Tract Infections

Advanced Science, Volume 13, Issue 19, 2 April 2026.
To address multidrug‐resistant urinary tract infections (MDR‐UTIs), we developed Cu‐ZIF8‐Ru nanozyme featuring an asymmetric Cu─N─Ru catalytic site. This unique structure enhances multienzyme‐mimetic activity, eradicating resistant bacteria by inducing a cuproptosis‐like death pathway through intracellular Cu2+ accumulation and energy depletion.
Guanlin Li, Shike Zhang, Jinggong Liu, Xin Wang, Heng Wang, Hongcan Yang, Yuhao Zhou, Wen Liu, Hongxing Liu, Wenqi Wu   +9 more
wiley   +1 more source

Quantitative study of H protein lipoylation of the glycine cleavage system and a strategy to increase its activity by co-expression of LplA

Journal of Biological Engineering, 2019
Glycine cleavage system (GCS) plays a key role in one-carbon (C1) metabolism related to the biosynthesis of a number of key intermediates with significance in both biomedicine and biotechnology.
Xinyi Zhang, Mei Li, Yingying Xu, Jie Ren, An-Ping Zeng   +4 more
doaj   +1 more source

Recessive MECR pathogenic variants cause an LHON-like optic neuropathy [PDF]

, 2023
Background: Leber's hereditary optic neuropathy (LHON) is a mitochondrial disorder characterised by complex I defect leading to sudden degeneration of retinal ganglion cells.
Bandello F., Barboni P., Baruffini E., Battista M., Caporali L., Carbonelli M., Carelli V., Cascavilla M. L., Degiorgi A., Fiorini C., La Morgia C., Maresca A., Ormanbekova D., Palombo F., Tropeano C. V.   +14 more
core   +1 more source

A Multi-Target Pharmacological Correction of a Lipoyltransferase LIPT1 Gene Mutation in Patient-Derived Cellular Models

Antioxidants
Mutations in the lipoyltransferase 1 (LIPT1) gene are rare inborn errors of metabolism leading to a fatal condition characterized by lipoylation defects of the 2-ketoacid dehydrogenase complexes causing early-onset seizures, psychomotor retardation ...
David Gómez-Fernández, Ana Romero-González, Juan M. Suárez-Rivero, Paula Cilleros-Holgado, Mónica Álvarez-Córdoba, Rocío Piñero-Pérez, José Manuel Romero-Domínguez, Diana Reche-López, Alejandra López-Cabrera, Salvador Ibáñez-Mico, Marta Castro de Oliveira, Andrés Rodríguez-Sacristán, Susana González-Granero, José Manuel García-Verdugo, José A. Sánchez-Alcázar   +14 more
doaj   +1 more source

PIK‐III‐Mediated Elevation of Thiamine Re‐Sensitises Renal Cell Carcinoma to Cuproptosis via Activating PDHA1

Cell Proliferation, Volume 59, Issue 3, March 2026.
PIK‐III enhancing the efficacy of cuproptosis to kill renal cancer cells through dysregulating thiamine metabolism and dephosphorylation of pyruvate dehydrogenase complex E1 (PDHA1), providing a potential option for treatment of cuproptosis‐resistant renal cancer by the combination of PIK‐III and elesclomol.
Dongdong Xie, Yu Wang, Wenjie Cheng, Minbo Yan, Kunyu Li, Xiang Wu, Jiaqing Wu, Zhuangzhuang Zhang, Yingbo Dai   +8 more
wiley   +1 more source

Different opinion on the reported role of Poldip2 and ACSM1 in a mammalian lipoic acid salvage pathway controlling HIF-1 activation. [PDF]

, 2018
Paredes et al recently described Poldip2 as a novel regulator of mitochondrial lipoylation through stabilisation of ACSM1 (1). We have several concerns with their proposed model based on the following reasons.Wellcome 102770/Z/13/Z and 205252/Z/16/Z ...
Bailey, Peter SJ, Dieckmann, Carol L, Hiltunen, J Kalervo, Kastaniotis, Alexander J, Nathan, James A   +4 more
core   +1 more source

NADH‐Reductive Stress Induced by Dihydrolipoamide Dehydrogenase Activation Contributes to Cuproptosis

Advanced Science, Volume 13, Issue 9, 13 February 2026.
This study demonstrates a cuproptosis mechanism involving nicotinamide adenine dinucleotide (NADH)‐reductive stress in neural cells. Copper activates dihydrolipoamide dehydrogenase under mitochondrial pH, accumulating NADH. Copper also induces mitochondrial permeability transition pore opening, facilitating NADH translocation to the cytosol and ...
Si‐Yi Zhang, Xing‐Hua Ren, Cheng‐Hong Zhang, Zhan‐You Wang   +3 more
wiley   +1 more source

The amidase domain of lipoamidase specifically inactivates lipoylated proteins in vivo.

PLoS ONE, 2009
BackgroundIn the 1950s, Reed and coworkers discovered an enzyme activity in Streptococcus faecalis (Enterococcus faecalis) extracts that inactivated the Escherichia. coli and E.
Maroya D Spalding, Sean T Prigge
doaj   +1 more source

Unravelling the lipoyl‐relay of exogenous lipoate utilization in Bacillus subtilis [PDF]

, 2019
Lipoate is an essential cofactor for key enzymes of oxidative and one-carbon metabolism. It is covalently attached to E2 subunits of dehydrogenase complexes and GcvH, the H subunit of the glycine cleavage system.
Ausubel F.M., Bachmann B.J., DeLano W.L., Morris T.W., Sambrook J., Sekiguchi J.   +5 more
core   +1 more source