Results 41 to 50 of about 20,316 (189)

Selenoprotein H Functions as a PPARα Coactivator to Link Selenium Homeostasis to Hepatic Lipid Metabolism and Protect against Steatohepatitis

Advanced Science, EarlyView.
Our study identifies selenium deficiency as a hallmark of MASH pathogenesis. Dietary selenium supplementation enhances hepatic fatty acid oxidation (FAO) and attenuates MASH progression by activating the PPARα pathway via selenoprotein H (SELENOH). This selenium‐SELENOH‐PPARα nexus redefines the functional scope of selenoproteins, moving from redox ...
Yuwei Zhang, Yuchen Wang, Binbin Li, Xin Li, Chenyu Liu, Yanhao Chen, Cheng Tian, Dongmei Wang, Xiaosong Gu, Chunping Jiang, Yuda Wei, Qiurong Ding   +11 more
wiley   +1 more source

Expanding the Phenotype of TUFM‐Related Combined Oxidative Phosphorylation Deficiency 4

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Combined oxidative phosphorylation deficiency 4 (COXPD4) is a rare mitochondrial condition caused by biallelic deleterious variants in the nuclear‐encoded gene TUFM. To date, most individuals with COXPD4 have presented with encephalopathy, hypotonia, and abnormal brain imaging. Many of the reported individuals died in infancy. We aim to expand
Noémie Villeneuve‐Cloutier, Jodi Warman‐Chardon, Danielle K. Bourque   +2 more
wiley   +1 more source

Altered Serum Amino Acid and Acylcarnitine Profiles in Hyperinsulinemic Hypoglycemia and Ketotic Hypoglycemia

Frontiers in Endocrinology, 2020
BackgroundIn addition to inborn metabolic disorders, altered metabolic profiles were reported to be associated with the risk and prognosis of some non-metabolic diseases, while as a rare metabolic disease, the overall secondary metabolic spectrum in ...
Zhen-Ran Xu, Xiao-Yi Zhu, Wei Lu, Wei-Hua Sun, Ruo-Qian Cheng, Jin-Wen Ni, Li Xi, Khalid Hussain, Fei-Hong Luo, Miao-Ying Zhang   +9 more
doaj   +1 more source

Acylcarnitines activate proinflammatory signaling pathways

American Journal of Physiology-Endocrinology and Metabolism, 2014
Incomplete β-oxidation of fatty acids in mitochondria is a feature of insulin resistance and type 2 diabetes mellitus (T2DM). Previous studies revealed that plasma concentrations of medium- and long-chain acylcarnitines (by-products of incomplete β-oxidation) are elevated in T2DM and insulin resistance.
Jennifer M, Rutkowsky, Trina A, Knotts, Kikumi D, Ono-Moore, Colin S, McCoin, Shurong, Huang, Dina, Schneider, Shamsher, Singh, Sean H, Adams, Daniel H, Hwang   +8 more
openaire   +3 more sources

Cytosolic Phosphoenoylpyruvate Carboxykinase Deficiency: Clinical, Biochemical, and Genetic Features of Five Non‐Finnish Patients

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Cytosolic phosphoenoylpyruvate carboxykinase (PEPCK‐C) is an essential, rate‐limiting enzyme in the gluconeogenesis pathway. PEPCK‐C deficiency presents with hypoglycaemia, hyperlactataemia and hepatopathy, and was first reported in association with bi‐allelic PCK1 variants in 2014.
Isaac Bernhardt, Polona Le Quesne Stabej, Claire Hart, Mark De Hora, Sarah Hulley, Mark Anderson, Harry G. Leitch, Hugh Lemonde, Bryony Ryder, James Davison   +9 more
wiley   +1 more source

Metabolic and Pharmacokinetic Profiling of a Ketone Ester by Background SGLT2 Inhibitor Therapy in HFrEF

JACC: Basic to Translational Science
Summary: Growing evidence supports therapeutic ketosis in heart failure with reduced ejection fraction, though uncertainty exists regarding use with SGLT2i and dose-dependent effects.
Senthil Selvaraj, MD, MS, MA, Lydia Coulter Kwee, PhD, Elizabeth J. Thompson, MD, Mengshu He, MS, Christoph P. Hornik, MD, PhD, Adam D. Devore, MD, Chetan B. Patel, MD, Robert J. Mentz, MD, Marat Fudim, MD, MHS, Lacey Taylor, MPH, Stephanie Milosovic, DPT, Melissa Hurdle, BS, William T. Cade, PT, PhD, Olga Ilkayeva, PhD, Michael J. Muehlbauer, PhD, Christopher B. Newgard, PhD, Daniel P. Kelly, MD, Payman Zamani, MD, MTR, Kenneth B. Margulies, MD, Svati H. Shah, MD, MS, MHS   +19 more
doaj   +1 more source

Acetylcarnitine Is Associated With Cardiovascular Disease Risk in Type 2 Diabetes Mellitus

Frontiers in Endocrinology, 2021
ObjectiveThis study aimed to identify the association between specific short-chain acylcarnitines and cardiovascular disease (CVD) in type 2 diabetes mellitus (T2DM).MethodWe retrieved 1,032 consecutive patients with T2DM who meet the inclusion and ...
Shuo Zhao, Shuo Zhao, Ming-Li Liu, Bing Huang, Fu-Rong Zhao, Ying Li, Xue-Ting Cui, Rong Lin   +7 more
doaj   +1 more source

Overview of Encapsulated Lysine and Methionine and Their Impacts on Transition Cow Performance and Health

Animal Research and One Health, EarlyView.
The transition period in dairy cows, spanning 3 weeks before and after calving, is a critical phase characterized by increased nutrient demands, reduced dry matter intake (DMI), and elevated risk of metabolic disorders such as negative nutrient balance (NNB), lipolysis, proteolysis, and oxidative stress.
Mohammed S. Seleem, Samy A. Elsaadawy, Zhaohai Wu, Lu Ma, Todd R. Callaway, Dengpan Bu   +5 more
wiley   +1 more source

NMR Characterization of Long-Chain Fatty Acylcarnitine Binding to the Mitochondrial Carnitine/Acylcarnitine Carrier

International Journal of Molecular Sciences, 2022
The mitochondrial carnitine/acylcarnitine carrier (CAC) transports short-, medium- and long-carbon chain acylcarnitines across the mitochondrial inner membrane in exchange for carnitine. How CAC recognizes the substrates with various fatty acyl groups, especially long-chain fatty acyl groups, remains unclear. Here, using nuclear magnetic resonance (NMR)
Ningning Zhang, Xiaopu Jia, Shuai Fan, Bin Wu, Shuqing Wang, Bo OuYang   +5 more
openaire   +2 more sources

Metabolic abnormalities and reprogramming in cats with naturally occurring hypertrophic cardiomyopathy

ESC Heart Failure, Volume 12, Issue 2, Page 1256-1270, April 2025.
Abstract Background and aims The heart is a metabolic organ rich in mitochondria. The failing heart reprograms to utilize different energy substrates, which increase its oxygen consumption. These adaptive changes contribute to increased oxidative stress.
Qinghong Li, Max Homilius, Erin Achilles, Laura K. Massey, Victoria Convey, Åsa Ohlsson, Ingrid Ljungvall, Jens Häggström, Brittany Vester Boler, Pascal Steiner, Sharlene Day, Calum A. MacRae, Mark A. Oyama   +12 more
wiley   +1 more source