Results 91 to 100 of about 4,222 (186)

The Preservation of Muscle Mitochondrial Machinery During Hypometabolic Hibernation in Scandinavian Brown Bears (Ursus arctos)

Acta Physiologica, Volume 242, Issue 4, April 2026.
ABSTRACT Aim Unlike humans, brown bears (Ursus arctos) uniquely preserve skeletal muscle mass and function during months of hibernation despite prolonged fasting and inactivity. We investigated how mitochondrial energetics respond in skeletal muscle to support this remarkable resilience.
Audrey Bergouignan, John Noone, Charlotte Brun, Laura Cussonneau, Alexandre Geffroy, Cecile Coudy‐Gandilhon, Isabelle Chery, Alina Lynn Evans, Jon Martin Arnemo, Jonas Kindberg, Guillemette Gauquelin‐Koch, Donal O'Gorman, Etienne Lefai, Fabrice Bertile   +13 more
wiley   +1 more source

Western blot validation and densitometric analysis of CDK1 and PDHA1 proteins.

, 2013
Confirmation of the expression profiles for CDK1 (A) and PDHA1 (B) was performed via immunoblot analysis. Densitometric analysis of the mean relative intensity (n = 3) for each target protein showed down-regulation of CDK1 by 1.42 fold (C) and up ...
Rohana Yusof (401395), Saiful Anuar Karsani (401397), Puteri Shafinaz Akmar Abdul-Rahman (401396), Christina Li-Ping Thio (401394)   +3 more
core   +1 more source

OMICS Profiling Identifies Signatures of Senescence in Osteogenesis Imperfecta Osteoblasts Counteracted by 4‐PBA

Journal of Cellular and Molecular Medicine, Volume 30, Issue 7, April 2026.
ABSTRACT Mutations in collagen I are the most common cause of osteogenesis imperfecta (OI), leading to delayed protein folding and structurally abnormal molecules. While some aberrant collagen is secreted into the extracellular matrix (ECM), impairing bone quality, a significant fraction is retained intracellularly, disrupting osteoblast homeostasis. 4‐
Roberta Besio, Elisa Maffioli, Erika Palladino, Alessandra Sala, Nadia Garibaldi, Valerio Izzi, Antonella Forlino, Gabriella Tedeschi   +7 more
wiley   +1 more source

T Cells Mediate Kidney Tubular Injury via Impaired PDHA1 and Autophagy in Type 1 Diabetes

, 2023
[[abstract]]Context: Nephropathy is a severe complication of type 1 diabetes (T1DM). However, the interaction between the PDHA1-regulated mechanism and CD4+ T cells in the early stage of kidney tubular injury remains unknown.
Chung-Hsing;Wang, Chung-Hsing;Lu, Wen-Li;Lu, Wen-Li;Ch, Shang-Lun;Chiang, Shang-Lun;Tsung-Hsun, Tsung-Hsun T;Tsai, Tsung-Hsun;Liu, Su-Ching;Liu, Su-Ching;Hs, Chia-Hung;Hsieh, Chia-Hung;Su, Pen-Hua;Su, Pen-Hua;黃志揚;Huang, Chih-Yang;Tsai, Fuu-Jen;Tsai, Fuu-Jen;Lin, Yu-Jung;Lin, Yu-Jung
core  

Regulation of pyruvate dehydrogenase complex: Dancing to different drums in cancer

International Journal of Cancer, Volume 158, Issue 6, Page 1464-1480, 15 March 2026.
Abstract Mechanisms governing the regulation of pyruvate dehydrogenase complex (PDC) are markedly modified in cancer cells compared to normal cells. PDC activity in normal cells is controlled by the reversible phosphorylation of three serine residues by dedicated kinases and phosphatases.
Mulchand S. Patel, Todd C. Rideout
wiley   +1 more source

X-linked pyruvate dehydrogenase complex deficiency due to a novel PDHA1 variant associated with structural brain abnormalities in a fetus

, 2023
We report a case of pyruvate dehydrogenase E1 alpha subunit deficiency associated with a novel hemizygous PDHA1 variant presenting prenatally as multiple structural brain abnormalities in a male fetus.
Antti M. Puhakka, Tanner, Laura M., Tynninen, Olli, Piippo, Kirsi, Puhakka, Antti M., Olli Tynninen, Laura M. Tanner, Kirsi Piippo   +7 more
core   +1 more source

Expert‐Designed Fact Sheets and AI‐Based Analysis of Patient Symptoms to Combat Diagnostic Delays in Inherited Metabolic Diseases

Journal of Inherited Metabolic Disease, Volume 49, Issue 2, March 2026.
ABSTRACT The importance of early diagnosis of inherited metabolic diseases (IMDs) is well known, as it allows early intervention to prevent or reduce complications and improve prognosis, since many of these disorders are treatable. However, diagnosis can still be delayed, and many patients remain undiagnosed. Reducing diagnosis delays is a primary goal
Aline Cano, Xiaoyi Chen, Azza Khemiri, Anais Brassier, Arnoux Jean‐Baptiste, Roseline Froissart, Juliette Bouchereau, Célia Hoebeke, Karin Mazodier, Bénédicte Héron, Philippe Labrune, Catherine Caillaud, David Cheillan, Yann Nadjar, Samia Pichard, Apolline Imbard, Magali Pettazzoni, Claire Douillard, Belmatoug Nadia, Anna‐Line Calatayud, Mounira Zerguini, Nicolas Garcelon, Jean‐François Benoist, Cécile Acquaviva, Pascale De Lonlay, the other members of the expert group consortium, Marie‐Thérèse Abi‐Warde, Cécile Acquaviva, Jean‐Baptiste Arnoux, Stéphanie Badiou, Magalie Barth, Nadia Belmatoug, Jean‐François Benoist, Juliette Bouchereau, Anais Brassier, Arnaud Bruneel, Catherine Caillaud, Aline Cano, Brigitte Chabrol, David Cheillan, Emmanuelle Corbe‐Guillard, Christelle Corne, Lena Damaj, Myriam Dao, Pascale De Lonlay, Anne‐Frédérique Dessein, Dries Dobbelaere, Claire Douillard, Thierry Dupré, François Feillet, Roseline Froissart, Margaux Gaschignard, Magali Gorce, Laurent Gouya, Anne‐Sophie Guemann, Bénédicte Héron, Célia Hoebeke, Apolline Imbard, Elsa Kaphan, François Labarthe, Philippe Labrune, Pascal Laforet, Thierry Levade, Elise Lebigot, Edouard Le Guillou, Olivier Lidove, Julien Maquet, Wladimir Mauhin, Clothilde Marbach, Karin Mazodier, Karine Mention, Fanny Mochel, Caroline Moreau, Yann Nadjar, Esther Noel, Mickael Obadia, Cécile Pagan, Magali Pettazzoni, Samia Pichard, Clement Pontoizeau, Aurélia Poujois, Isabelle Redonnet‐Vernhet, Frédérique Sabourdy, Manuel Schiff, Christine Serratrice, Aude Servais, Caroline Sevin, Anne Spraul, Bénédicte Sudrié, Marine Tardieu, Sandrine Vuillaumier, Camille Wicker, Arnaud Wiedemann‐Fode, Vincent Barlogis, Nathalie Boddaert, Kanetee Busiah, Annabelle Chaussenot, Dominique Debray, Céline Falaise, Muriel Girard, Dalila Habes, Annie Harroche, Florence Lacaille, Mehdi Oualha, Caroline Ovaert, Rachel Reynaud, Caroline Rousset‐Rouvière, Cécile Rouzier, Karim Wahbi   +108 more
wiley   +1 more source

Protein Lactylation in Cancer: Mechanisms and Therapeutic Targets

MedComm, Volume 7, Issue 3, March 2026.
This schematic illustrates the central role of lactylation, a lactate‐derived posttranslational modification, in linking metabolic reprogramming to cancer progression and therapy resistance. At its core, lactylation modulates proteins, influenced by metabolic shifts and environmental factors.
Qianying Ouyang, Qianyu Hu, Caiqin Wang, Yizi He, Ruolan Zeng, Yajun Li, Chang Su, Guige Lu, Xueting Zhu, Ling Xiao, Hui Zhou   +10 more
wiley   +1 more source

Mild Phenotype in a Male with Pyruvate Dehydrogenase Complex Deficiency Associated with Novel Hemizygous In-Frame Duplication of the E1α Subunit Gene (PDHA1) [PDF]

, 2014
Pyruvate dehydrogenase complex (PDHC) deficiency is an inborn error of metabolism that occurs most commonly due to mutations in the X-linked E1α subunit gene (PDHA1).
Kimonis, VE, Gargus, JJ, Hasso, AN, Steller, J, Gibbs, LH   +4 more
core   +1 more source

Association of Phosphorylated Pyruvate Dehydrogenase with Pyruvate Kinase M2 Promotes PKM2 Stability in Response to Insulin

, 2023
Insulin is a crucial signalling molecule that primarily functions to reduce blood glucose levels through cellular uptake of glucose. In addition to its role in glucose homeostasis, insulin has been shown to regulate cell proliferation.
Hwee-Seon Park, Jong-Il Kim, Vikas Kumar, Keun Woo Lee, Jong-Bok Seo, Jae-Bong Park, Rokibul Islam, Abu Jubayer Hossain   +7 more
core   +1 more source