Results 31 to 40 of about 3,572 (156)

Partial complementation of pyruvate dehydrogenase deficiency by independently expressed lipoyl and catalytic domains of the dihydrolipoamide acetyltransferase component [PDF]

FEMS Microbiology Letters, 1989
Two compatible plasmids encoding a hybrid lipoyl domain and a defective pyruvate dehydrogenase (PDH) complex which lacks lipoyl domains, were co-expressed in a strain of Escherichia coli deleted for the PDH complex genes. In vivo complementation between the mutant complexes and the independent lipoyl domains was observed using growth tests in liquid ...
G C, Russell, R A, Williamson, J R, Guest   +2 more
openaire   +2 more sources

A metabolic model of the mitochondrion and its use in modelling diseases of the tricarboxylic acid cycle. [PDF]

, 2011
BACKGROUND: Mitochondria are a vital component of eukaryotic cells and their dysfunction is implicated in a large number of metabolic, degenerative and age-related human diseases.
Robinson, Alan J, Smith, Anthony C
core   +4 more sources

3-Hydroxyisobutyryl-CoA hydrolase involved in isoleucine catabolism regulates triacylglycerol accumulation in Phaeodactylum tricornutum [PDF]

, 2017
Since methylmalonyl-CoA epimerase appears to be absent in the majority of photosynthetic organisms, including diatoms, (S)-methylmalonyl-CoA, the intermediate of isoleucine (Ile) catabolism, cannot be metabolized to (R)methylmalonyl-CoA then to succinyl ...
Gong, Yangmin, Hu, Hanhua, Li, Xiaolong, Pan, Yufang, Yang, Juan   +4 more
core   +1 more source

Ferroptosis-Related Flavoproteins: Their Function and Stability [PDF]

, 2021
Ferroptosis has been described recently as an iron-dependent cell death driven by peroxidation of membrane lipids. It is involved in the pathogenesis of a number of diverse diseases.
Vabulas, R. Martin
core   +1 more source

Mitochondrial alpha-ketoglutarate dehydrogenase complex generates reactive oxygen species [PDF]

, 2004
Mitochondria-produced reactive oxygen species (ROS) are thought to contribute to cell death caused by a multitude of pathological conditions. The molecular sites of mitochondrial ROS production are not well established but are generally thought to be ...
Beal, M. Flint, Browne, SE., Chinopoulos, Christos, Fiskum, G., Lorenzo, BJ., Patel, Mulchand S., Starkov, Anatoly A.   +6 more
core   +1 more source

The natural history of dihydrolipoamide dehydrogenase deficiency in Israel

Journal of Inherited Metabolic Disease
AbstractDihydrolipoamide dehydrogenase (DLD) deficiency is an ultra‐rare autosomal‐recessive inborn error of metabolism, affecting no less than five mitochondrial multienzyme complexes. With approximately 30 patients reported to date, DLD deficiency was associated with three major clinical presentations: an early‐onset encephalopathic phenotype with ...
Ben Pode‐Shakked, Yuval E. Landau, Nava Shaul Lotan, Joshua Manor, Nitsan Haham, Eyal Kristal, Eli Hershkovitz, Guy Hazan, Yarden Haham, Shlomo Almashanu, Yair Anikster, Orna Staretz‐Chacham   +11 more
openaire   +2 more sources

Neurological, Psychiatric, and Biochemical Aspects of Thiamine Deficiency in Children and Adults. [PDF]

, 2019
Thiamine (vitamin B1) is an essential nutrient that serves as a cofactor for a number of enzymes, mostly with mitochondrial localization. Some thiamine-dependent enzymes are involved in energy metabolism and biosynthesis of nucleic acids whereas others ...
Adams, Alfadhel, Alfadhel, Alfadhel, Aljabri, Anwar, Aronson, Arts, Attwell, Baio, Banka, Barennes, Benton, Bettendorff, Bettendorff, Biesecker, Brown, Butterworth, Butterworth, Butterworth, Calhau, Carney, Chandrakumar, Chen, Cheung, Chornyy, Chugani, Colbert, Croft, Day, De Meirleir, Fattal-Valevski, Fattal-Valevski, Fleming, Forouhi, Foulon, Foulon, Fraccascia, Fraccascia, Fraser, Frustaci, Fujita, Galvin, Ganapathy, Gangolf, Ganie, Ghaleiha, Gibson, Giulivi, Gold, Greenwood, Gu, Harel, Hiffler, Hilker, Inaba, Ishaque, Israels, Jankowska-Kulawy, Junior, Kamasak, Karabatsiakis, Kelley, Kerns, Khaw, Kitamura, Kornreich, Kril, Laforenza, Leichter, Lindhurst, Liu, Lonsdale, Lonsdale, Lonsdale, Lorber, Lu, Lukienko, Luxemburger, Lương, Ma, Magos, Mahajan, Mahmood, Martel, Martin, Mayr, McLure, Mergenthaler, Mezzar, Mimouni-Bloch, Molina, Mulholland, Mulle, Muoio, Murata, Nabokina, Nabokina, Nardone, Navarro, Nikseresht, Obrenovich, Oishi, Opdenakker, Ortega Garcia, Ortigoza-Escobar, Pepersack, Perez-Duenas, Plaitakis, Prabakaran, Prohaska, Rabie, Randle, Renthal, Rindi, Rindi, Robinson, Roust, Rubio-Lopez, Said, Schaller, Scrimshaw, Sequeira Lopes da Silva, Shi, Singleton, Siu, Skjeldal, Smidt, Society, Song, Sperringer, Spiegel, Stargrove, Subramanian, Subramanian, Sweet, Tabarki, Talwar, Thomson, Thornalley, van den Brink, Vasconcelos, Vernau, Vernau, Vimokesant, Watkins, Wendel, Whitfield, Whitfield, Wierzbicki, Williams, Yang, Zhang, Zhao, Zuccoli   +154 more
core   +2 more sources

Nano Delivery Strategy Opens Effective Avenues for the Treatment of Colorectal Cancer

Medicine Bulletin, EarlyView.
ABSTRACT Colorectal cancer (CRC) presents significant therapeutic challenges because of chemoresistance, systemic toxicity, and the complexity of the tumor microenvironment (TME). Nanodrug delivery systems (NDDS) have emerged as versatile platforms capable of overcoming these limitations by enhancing tumor accumulation, improving pharmacokinetics, and ...
Qiurong Wei, Yunli Xu, Tianjiao Zhang, Shuyu Xu, Haonan Wang, Yuan Wang, Xinxin Xie, Xiaolong Liang   +7 more
wiley   +1 more source

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

Cell Proliferation, EarlyView.
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

The Complex Relation of Branched‐Chain Amino Acids and Inflammation in the Obesity and Diabetes Context

Obesity Reviews, EarlyView.
ABSTRACT In a scenario with increasing cases of obesity and diabetes worldwide, branched‐chain amino acids (BCAA) metabolism has become an important factor in the understanding of these pathologies. More recently, its chronic high plasma levels have been postulated, alongside glucose, inflammatory factors, and other molecules, as an important ...
Bernardo Starling‐Soares, Monique Macedo Coelho, Bruna Guerra Campolina, Camila Kümmel Duarte, Tatiani Uceli Maioli   +4 more
wiley   +1 more source