Results 201 to 210 of about 33,228 (280)

Elamipretide Improves Mitochondrial Function in Mitochondrial Trifunctional Protein‐Deficient Mice and Human Fibroblasts

Journal of Inherited Metabolic Disease, Volume 49, Issue 1, January 2026.
ABSTRACT Mitochondrial trifunctional protein (TFP) deficiency is an inherited disorder of long‐chain fatty acid β‐oxidation (FAO). TFP is a heteromeric enzyme composed of two α and two β‐subunits. Despite early detection and dietary treatment, TFP deficiency patients often develop hypoglycemia, rhabdomyolysis, cardiomyopathy, and peripheral neuropathy.
Eduardo Vieira Neto, Meicheng Wang, Austin J. Szuminsky, Lethicia Ferraro, Shakuntala Basu, Xuejun Zhao, Anuradha Karunanidhi, Yudong Wang, Jerry Vockley   +8 more
wiley   +1 more source

Systemic Primary Carnitine Deficiency Presenting With Substantia Nigra and Basal Ganglia Injury: A Case Report. [PDF]

JIMD Rep
Saito T, Soma K, Kashisaka M, Iketani K, Matsumoto M, Ueda T, Nishiyama M, Maruyama A, Nakai R, Sakihama H, Kurosawa H, Morisada N, Kobayashi H, Ozaki K.   +13 more
europepmc   +1 more source

Energy Metabolism Under Stress: Late‐Stage Leigh Syndrome Reveals Profound Cardiometabolic Perturbations in Ndufs4 KO Mice

Journal of Inherited Metabolic Disease, Volume 49, Issue 1, January 2026.
Cardiac bioenergetic and metabolic perturbations in late‐stage Leigh syndrome. Whole‐body NDUFS4 loss severely impairs cardiac complex I activity and respiration, shifting reliance to complex II and reducing glycolytic, TCA, and amino acid‐derived energy‐generating substrates in the heart.
Karin Terburgh, Nastassja Sweeney, Roan Louw   +2 more
wiley   +1 more source

Navigating Solute Carrier Transporters—A Comprehensive Review of Functionalized Small Molecule Probes for Target Identification and Characterization

Medicinal Research Reviews, Volume 46, Issue 1, Page 238-271, January 2026.
ABSTRACT Solute carrier transporters (SLCs) are integral membrane proteins that play pivotal roles in maintaining cellular homeostasis by mediating the transport of a diverse range of substrates across cell membranes. With their involvement in fundamental physiological processes such as nutrient uptake, neurotransmitter signaling, and drug transport ...
Majlen A. Dilweg, Tracie Widjaja, Adriaan P. IJzerman, Daan van der Es   +3 more
wiley   +1 more source

Partial Replacement of Maltodextrin With Galactose in Post‐Weaning Diets of Mice Alters Body Composition and Energy Metabolism in Early‐Life and Programs Hepatic Igf1 Expression in Later‐Life

Molecular Nutrition &Food Research, Volume 70, Issue 1, January 2026.
Galactose‐containing post‐weaning diets given for 3 weeks decreased body weight and changed energy metabolism compared to a maltodextrin‐enriched control diet in the early life of mice. After following 9 weeks of a high‐fat diet intervention, the mice fed in the early life the galactose‐containing diets had no obvious nutritional programming effects ...
Peixin Sun, Maryam Rakhshandehroo, Melissa Bekkenkamp‐Grovenstein, Lidewij Schipper, Jaap Keijer, Evert M. van Schothorst   +5 more
wiley   +1 more source

Screening primary carnitine deficiency in 10 million Chinese newborns: a systematic review and meta-analysis. [PDF]

Orphanet J Rare Dis
Zhou J, Li G, Zeng Y, Qiu X, Zhao P, Huang T, Wang X, Luo J, Lin N, Xu L.   +9 more
europepmc   +1 more source

The Effects of Source and Concentration of Digestible Methionine + Cystine in Diet on Productive Performance, Egg Quality Traits and Blood Metabolites in Commercial Laying Hens

Veterinary Medicine and Science, Volume 12, Issue 1, January 2026.
Hens fed diets with digestible Met + Cys levels equal to (0.742% of diet) or exceeding the strain recommendation (0.929%) exhibited higher egg production compared to 0.555% Met + Cys level. Approximately 0.929% dietary level of digestible Met + Cys enhanced egg mass, egg weight and daily feed intake, and improved feed conversion ratio.
Saeed Ghavi, Ahmad Hassanabadi, Heydar Zarghi   +2 more
wiley   +1 more source

The Gut Microbiota–Insulin Resistance Axis: Mechanisms, Clinical Implications, and Therapeutic Potential

FASEB BioAdvances, Volume 8, Issue 1, January 2026.
Dysbiosis‐induced alterations in microbial metabolite production promote intestinal barrier disruption and systemic inflammation, driving insulin resistance and metabolic dysfunction. Microbiota‐targeting therapies represent promising approaches to restore host metabolic health.
Mohamad Al Qassab, Nadim Chaarani, Amira Hamou, Rasha Harb, Ahmad Jradi, Mira Zeineddine, Hilda E. Ghadieh, Ziad Abi Khattar, Sami Azar, Amjad Kanaan, Frederic Harb   +10 more
wiley   +1 more source

Effects of including white clover and plantain species in perennial ryegrass pastures on qualitative indicators and metabolome of milk

International Journal of Dairy Technology, Volume 79, Issue 1, January–March 2026.
Mixed sward pasture systems are being investigated due to their potential to improve bio diversity and sustainability metrics; our study provides a due diligence on the impact of pastures containing perennial ryegrass, white clover and plantain species on the composition and functionality of milk.
Nisha J Suthar, Eoin Wims, Michael Dineen, Raghunath Pariyani, Denis Lynch, Lorraine M Bateman, David T Mannion, Anita R Maguire, Kieran N Kilcawley, James A O'Mahony, Tom F O'Callaghan   +10 more
wiley   +1 more source

NRF2 signalling in cytoprotection and metabolism

British Journal of Pharmacology, Volume 183, Issue 1, Page 101-114, January 2026.
The KEAP1‐NRF2 system plays a central role in cytoprotection in defence mechanisms against oxidative stress. The KEAP1‐NRF2 system has been regarded as a sulfur‐utilizing cytoprotective mechanism, because KEAP1 serves as a biosensor for electrophiles by using its reactive thiols and NRF2 is a transcriptional factor regulating genes involved in sulfur ...
Shohei Murakami, Yusuke Kusano, Keito Okazaki, Takaaki Akaike, Hozumi Motohashi   +4 more
wiley   +1 more source