Results 61 to 70 of about 3,959 (161)

Inhibition of phosphomannose isomerase by fructose 1-phosphate: an explanation for defective N-glycosylation in hereditary fructose intolerance. [PDF]

, 1996
Isoelectrofocusing of serum sialotransferrins from patients with untreated hereditary fructose intolerance (HFI) shows a cathodal shift similar to that in carbohydrate-deficient glycoprotein (CDG) syndrome type I and in untreated galactosemia.
Pirard, M, Michel Pirard, Ewa Pronicka, Emile Van Schaftingen, Jaeken, J, Adamowicz, M, Pronicka, E, Jaak Jaeken, Maciej Adamowicz, Van Schaftingen, Emile   +9 more
core   +1 more source

Excessive training does not induce mitochondrial dysfunction or impair insulin signalling within skeletal muscle

The Journal of Physiology, EarlyView.
Abstract figure legend Three weeks of overtraining in trained endurance athletes reduced exercise performance without impairing glucose tolerance. In skeletal muscle, overtraining was associated with increased expression of lipid metabolism‐related proteins, enhanced mitochondrial biogenesis and preserved insulin signalling, despite elevated oxidative ...
Geneviève J. DesOrmeaux, Henver S. Brunetta, Pierre‐Andre Barbeau, Alexandra M. Coates, Christopher Pignanelli, Fasih A. Rahman, Jamie F. Burr, Graham P. Holloway   +7 more
wiley   +1 more source

Hereditary fructose intolerance in a patient with phenylketonuria [PDF]

, 1991
Classical phenylketonuria (PKU) and hereditary fructose intolerance (HFI) are two inborn errors of metabolism that have an autosomal recessive mode of inheritance. In this paper, we described a 3-year-old girl with PKU and HFI.
T Coşkun, I Ozalp, G Tekinalp
core  

Lipidomics uncovers metabolic manifestations related to liver steatosis and low-grade systemic inflammation in diet-treated hereditary fructose intolerance patients.

Clinical Nutrition
BACKGROUND AND AIMS Hereditary Fructose Intolerance (HFI), a rare autosomal recessive metabolic disorder, has historically been considered benign when treated with a lifelong fructose-, sucrose and sorbitol-restricted diet.
Javier Heras, María Unceta Suárez, Maria Mercado-Gómez, Leire Uraga Gracianteparaluceta, Xavier Buqué, Cristina Alonso, Marina Serrano‐Maciá, C. Alcalde, A. Cano, I. González-Recio, Naroa Goikoetxea‐Usandizaga, Edorta Mora Ballesteros, Amaya Belanger-Quintana, Elvira Cañedo-Villarroya, L. Ceberio, Silvia Chumillas-Calzada, Patricia Correcher, Dolores García-Arenas, Igor Gómez, Tomás Hernández, E. Izquierdo-García, Dámaris Martínez Chicano, Montserrat Morales, C. Pedrón-Giner, E. P. Jáuregui, L. Peña-Quintana, P. Sánchez-Pintos, J. Serrano-Nieto, Irene Serrano-Gonzalo, I. V. Miñana, J. Larena, María-Luz Couce, M. Martínez‐Chantar, Patricia Aspichueta, Teresa C. Delgado   +34 more
semanticscholar   +1 more source

Molecular insights and translational opportunities to enhance heat tolerance in rice

The Plant Genome, Volume 19, Issue 3, September 2026.
Abstract Heat stress is an increasingly serious threat to rice (Oryza sativa L.) productivity, yet the genetic and regulatory architecture underlying thermotolerance remain poorly resolved and fragmented across studies. Earlier research focused on individual pathways or specific developmental stages; however, recent advances now support an integrated ...
Prabhat Rana, Chanderkant Chaudhary, Rajat Pruthi, Mansi Sharma, Bhupinderjeet Singh, Ravi Kiran Reddy Kondi, Mallesham Bulle, Prasanta K. Subudhi   +7 more
wiley   +1 more source

Northeast China Traditional Kimchi (Paocai) Probiotic Resources Metabolized Uric Acid and Relieved Hyperuricemia in Mice

eFood, Volume 7, Issue 4, August 2026.
Traditional fermented Kimchi “paocai”, commonly consumed in Northeast China, was screened for probiotic resources capable of degrading uric acid in vitro and alleviating hyperuricemia in a mouse model. Abstract Current pharmacological therapies for hyperuricemia (HUA), though effective, pose safety concerns, including allopurinol‐induced severe dermal ...
Aman Khan, Xiaoyu Wang, Binyu Cui, Xiaoqian Zhou, Yingying Lu, Lifang Ma, Qizhuang Li, Dechun Yin, Weidong Wang   +8 more
wiley   +1 more source

Glucose Transporter 1 in Health and Disease

MedComm, Volume 7, Issue 8, August 2026.
As the quintessential facilitator of basal glucose uptake, glucose transporter 1 (GLUT1) is indispensable for maintaining systemic energy homeostasis. This graphical abstract delineates the multidimensional landscape of GLUT1 in normal physiology. It highlights its tissue‐specific metabolic roles—from fueling erythrocytes and fetal development to ...
Yi Tai, Zhihao Chen, Yuping Zhu, Weidong Han   +3 more
wiley   +1 more source

Review of nutrition management of pediatric intestinal pseudo‐obstruction

Nutrition in Clinical Practice, Volume 41, Issue 4, Page 1048-1060, August 2026.
Abstract Chronic intestinal pseudo‐obstruction (CIPO) is a rare, heterogeneous, and debilitating disorder characterized by profound intestinal dysmotility and severe nutrition challenges. Its presentation resembles that of mechanical bowel obstruction, but CIPO occurs in the absence of luminal obstruction.
Senthilkumar Sankararaman, Raisa Rani James, Bushra El‐Amaireh, Andrea Adler, Kadakkal Radhakrishnan, Sujithra Velayuthan   +5 more
wiley   +1 more source

Report of the Scientific Committee of the Spanish Agency for Food Safety and Nutrition (AESAN) on Hereditary Fructose Intolerance (HFI), or aldolase B deficiency, and fructose malabsorption (intestinal fructose intolerance)

Food Risk Assess Europe
Hereditary Fructose Intolerance (HFI), also known as aldolase B deficiency, is an inherited metabolic disorder caused by the deficiency of that enzyme, which participates in the fructose metabolism in the liver, kidneys and small intestine.
Araceli Díaz Perales, Irene Bretón Lesmes, Ángel Gil Izquierdo, Gema Nieto Martínez, Silvia Pichardo Sánchez, María de Cortes Sánchez Mata, María Ángeles Carlos Chillerón   +6 more
semanticscholar   +1 more source

Differential Pathophysiological Drivers of Susceptibility to Type 2 Diabetes and Metabolic Dysfunction–Associated Steatotic Liver Disease: Ethnic Differences in Insulin Dynamics, Whole‐Body Fat Metabolism, and Organ‐Specific Lipid Deposition

Obesity Reviews, Volume 27, Issue 8, August 2026.
ABSTRACT Introduction This narrative review explores the epidemiological evidence and potential underlying pathophysiological defects underlying the disproportionately greater risk of Type 2 diabetes (T2D) and cardiometabolic disease in people of South Asian and African Caribbean ancestry compared with White Europeans.
Daniel J. Cuthbertson, Martin Whyte, Alex E. Henney, Uazman Alam, Louise Goff, Barbara A. Fielding, A. Margot Umpleby   +6 more
wiley   +1 more source