Results 11 to 20 of about 1,169 (119)

The Gut Commensal Butyricimonas Virosa Modulates Gut Microbiota‐Dependent Thiamine Metabolism and Attenuates Mouse Steatotic Liver Disease [PDF]

Advanced Science
Metabolic dysfunction‐associated steatotic liver disease (MASLD) is a common chronic liver disease. This study investigates the anti‐MASLD effects of dietary prebiotic stachyose (STA) on disease progression identifying Butyricimonas virosa as a key ...
Ningning He, Haoyu Wang, Zizhen Yang, Hui Li, Bei Liu, Kaiwei Chen, Zhinan Wu, Xinnan Zhao, Hewei Liang, Mengmeng Wang, Xiaofang Li, Yiyi Zhong, Haifeng Zhang, Liang Xiao, Karsten Kristiansen, Jixing Peng, Yuanqiang Zou, Shangyong Li   +17 more
doaj   +3 more sources

Clinical Profiles, Genetic Variants, and Neurodevelopmental Outcomes Following Liver Transplantation in Maple Syrup Urine Disease: A Study From Palestine. [PDF]

JIMD Rep
ABSTRACT Maple syrup urine disease (MSUD) is a rare, autosomal recessive metabolic disorder resulting from a deficiency of the branched‐chain α‐ketoacid dehydrogenase complex. This leads to the accumulation of branched‐chain amino acids and their corresponding ketoacids, causing acute metabolic crises and progressive neurological damage if untreated ...
Khalaf-Nazzal R, Haj-Ahmad H, Zaid J, AbuShamleh M, Dweikat I.   +4 more
europepmc   +2 more sources

The Complex Relation of Branched-Chain Amino Acids and Inflammation in the Obesity and Diabetes Context. [PDF]

Obes Rev
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 ...
Starling-Soares B, Coelho MM, Campolina BG, Duarte CK, Maioli TU.   +4 more
europepmc   +2 more sources

Mitochondria-Related Pathogenic Genes in Paediatric Asthma: A Multi-Omics Mendelian Randomization Study. [PDF]

J Cell Mol Med
ABSTRACT Mitochondrial dysfunction is implicated in asthma pathogenesis, but causal roles of mitochondrial‐related genes in paediatric asthma remain unclear. We performed a multi‐omics Mendelian randomization study integrating GWAS data from paediatric asthma cohorts with blood‐based methylation quantitative trait loci (mQTLs), expression QTLs (eQTLs ...
Zhang B, Li Y, Ding B, Li X, Lu Y.
europepmc   +2 more sources

PRSS55 regulates BCAA metabolism and interacts with BCKDK and BCKDHA in mouse testes and sperm [PDF]

Cell & Bioscience
Backgrounds Recent research has emphasized the significance of testis-specific serine proteases in regulating various aspects of sperm maturation and function.
Haoyang Ge, Chunling Shen, Wenfeng Xiong, Shunyuan Lu, Chaojie Li, Lingyun Tang, Yan Shen, Hongxin Zhang, Jian Fei, Zhugang Wang   +9 more
doaj   +2 more sources

Sodium-glucose cotransporter 2 inhibitors—but not insulin—enhance renal branched-chain amino acid catabolism [PDF]

Frontiers in Endocrinology
Aims/hypothesisSodium–glucose cotransporter 2 inhibitors (SGLT2i) confer cardio-renal protection, and recent work implicates enhanced branched-chain amino acid (BCAA) catabolism as a potential mechanism in the heart.
Moeko Sakamoto, Nao Hasuzawa, Lixiang Wang, Kyoko Tashiro, Miyuki Kitamura, Shimpei Iwata, Nobuhiko Wada, Seiji Nomura, Sawako Moriyama, Mizuki Gobaru, Yukihiro Inoguchi, Ayako Nagayama, Kenji Ashida, Yoshinori Moriyama, Masatoshi Nomura   +14 more
doaj   +2 more sources

Regional Differences in Cardiac Marker Gene Expression and Branched-Chain Amino Acid Metabolism in the Bovine Heart [PDF]

Animals
Although the hearts of large animals are known to exhibit regional heterogeneity in structure, function, and metabolism, their spatial patterns of gene expression remain poorly understood.
Rin Takiguchi, Kenichi Watanabe, Yutsuki Doai, Misuzu Hashimoto, Hiroyuki Watanabe, Yuki Muranishi   +5 more
doaj   +2 more sources

Adenine base editing rescues disrupted BCKDH function and reduces BCAAs toxic accumulation in maple syrup urine disease patient iPSC-hepatic organoids [PDF]

Stem Cell Research & Therapy
Background Maple syrup urine disease (MSUD) is an inherited metabolic disorder caused by a deficiency in the activity of the hepatic branched-chain α-ketoacid dehydrogenase (BCKDH) complex, which leads to the toxic accumulation of three branched-chain ...
Haiyan Zhang, Zirui Wan, Xiaomei Li, Bin Wang, Jingyun Guan, Yue Li, Xiaohua Jin, Xu Ma, Guohua Liu   +8 more
doaj   +2 more sources

Expanding the genotypic and phenotypic spectrum of Egyptian children with maple syrup urine disease [PDF]

Scientific Reports
Maple Syrup Urine Disease (MSUD, OMIM# 248600) is an autosomal recessive inborn error of metabolism characterized by elevated branched chain amino acids (BCAA) leucine/isoleucine and valine in blood of affected children.
Zeinab S. Abdelkhalek, Shadia M. Hussein, Iman G. Mahmoud, Areef Ramadan, Mona A. Kamel, Marian Y. Girgis, Mohamed A. Elmonem   +6 more
doaj   +2 more sources

Altered branched chain ketoacids underlie shared metabolic phenotypes in type 1 diabetes and maple syrup urine disease [PDF]

Communications Medicine
Background Diabetic ketoacidosis is an acute, potentially life-threatening, metabolic complication and often first presentation of type 1 diabetes (T1D) mellitus. Here, we investigated the metabolic and lipid profiles from pediatric patients with T1D, at
Domenico Roberti, Abby L. Grier, Julie A. Reisz, Fara Vallefuoco, Alicia Key, Shaun Bevers, Monika Dzieciatkowska, Travis Nemkov, Marcella Contieri, Angela Zanfardino, Philip J. Norris, Michael P. Busch, Vienna Kauffman, Holmes D. Morton, Eric J. Earley, Grier P. Page, Pierluigi Marzuillo, Angelo D’Alessandro   +17 more
doaj   +2 more sources