Results 21 to 30 of about 4,895 (172)

Novel Role of Gut-Derived Roseburia Intestinalis in Safeguarding Intestinal Barrier Integrity and Microenvironment Homeostasis During Arsenic Exposure. [PDF]

Adv Sci (Weinh)
Arsenic exposure disrupts intestinal barriers and gut microenvironment. Fecal microbiota transplantation (FMT) alleviates arsenic‐induced damage, with gut‐derived Roseburia intestinalis (R.i) identified as a key protective strain. R.i administration counters arsenic toxicity through immunomodulatory pathways and metabolites.
Zhou L, Wang C, Gao J, Wu X, Li G, Jiang X, Xia Y, Zhang J, Lv B, Zhao F, Zhang H, Pi H, Qiu J, Xu S, Zou Z, Chen C.   +15 more
europepmc   +2 more sources

Late-Onset Glycogen Storage Disease Type II (Pompe’s Disease) with a Novel Mutation: A Malaysian Experience

Case Reports in Neurological Medicine, 2014
Pompe’s disease (acid maltase deficiency, glycogen storage disease type II) is an autosomal recessive disorder caused by a deficiency of lysosomal acid α-1,4-glucosidase, resulting in excessive accumulation of glycogen in the lysosomes and cytoplasm of ...
Hiew Fu Liong, Siti Aishah Abdul Wahab, Yusnita Yakob, Ngu Lock Hock, Wong Kum Thong, Shanthi Viswanathan   +5 more
doaj   +1 more source

Comparative Study of Acid Maltase Deficiency: Biochemical Differences Between Infantile, Childhood, and Adult Types

, 1972
Maltase (α-1,4-glucosidase) activity was determined at various pH values in tissues in infantile (heart, liver, skeletal muscle), childhood (heart, liver, skeletal muscle), and adult (liver, skeletal muscle, leukocytes) types of acid maltase deficiency ...
Angelini C., Engel A. G.
core   +2 more sources

Infantile-onset Pompe disease: clinical case, diagnostic challenges and analysis of the effectiveness of enzyme replacement therapy

Zdorovʹe Rebenka
Background. Pompe disease (PD) is a genetic orphan progressive multisystem neuromuscular disorder that belongs to the group of lysosomal storage diseases and often leads to death. The only treatment available is lifelong enzyme replacement therapy (ERT).
N.V. Shyshkanova, M.V. Miroshnychenko, N.V. Samonenko, V.H. Ivanova, O.O. Dziuba, N.V. Olkhovych, Yu.A. Chornyi, L.V. Radchenko, E.V. Berezhna, V.V. Sokolenko, D.O. Kaminchenko   +10 more
doaj   +1 more source

Survival and associated factors in 268 adults with Pompe disease prior to treatment with enzyme replacement therapy

Orphanet Journal of Rare Diseases, 2011
Background Pompe disease is a rare lysosomal storage disorder characterized by muscle weakness and wasting. The majority of adult patients have slowly progressive disease, which gradually impairs mobility and respiratory function and may lead to ...
Reuser Arnold JJ, Hop Wim CJ, de Vries Juna M, Güngör Deniz, van Doorn Pieter A, van der Ploeg Ans T, Hagemans Marloes LC   +6 more
doaj   +1 more source

SuFEx‐Enabled Reprogramming of Flavonoids for Selective α‐Glucosidase Covalent Inhibition

Advanced Science, EarlyView.
Selective inhibition of intestinal α‐glucosidase remains limited by poor enzyme specificity and off‐target metabolic effects. Here, SuFEx click chemistry is used to reprogram natural flavonoids into covalent inhibitors with enhanced α‐glucosidase selectivity and controlled reactivity. This strategy enables effective regulation of carbohydrate digestion
Fengyu Guo, Liwei Zhang, Minlong Wang, Yanjin Du, Tianyu Zhang, Hao Chen, Jiazeng Sun, Yang Yu, Zude He, Jie An, Xiaoxu Zhang, Weilin Lin, Fazheng Ren, Pengjie Wang, Ping Liu   +14 more
wiley   +1 more source

Integration of Nutrition and Organic Agriculture Framework in Managing Gastrointestinal Nematodes in Ruminants

Animal Research and One Health, EarlyView.
Gastrointestinal nematode infections damage the gastrointestinal epithelial tissues of ruminants, affecting nutrient utilization and overall production performance. This review outlines host‐gastrointestinal nematode interactions and discusses integrated control strategies, including nutritional supplementation, grazing management, vaccines, and ...
Wenxun Chen, Xiangfang Tang, Yimin Zhuang, Shengjun Zhao, Ying Ren, Xiaokang Lv   +5 more
wiley   +1 more source

Pompe Disease: Early Diagnosis and Early Treatment Make a Difference

Pediatrics and Neonatology, 2013
Pompe disease (glycogen storage disease type II or acid maltase deficiency) is a lysosomal disorder in which acid α-glucosidase (GAA) deficiencies lead to intralysosomal accumulation of glycogen in all tissues; most notably in skeletal muscles.
Yin-Hsiu Chien, Wuh-Liang Hwu, Ni-Chung Lee   +2 more
doaj   +1 more source

Combining phenotypic, physiological, and multi‐omics studies to provide new insights on the role of 2,4‐epibrassinolide in regulation of seed shattering in Elymus sibiricus L.

Grassland Research, EarlyView.
Abstract Background Seed shattering limits the production of Elymus sibiricus L., and application of exogenous brassinosteroid significantly alleviates plant organ abscission. Methods To explore the potential regulatory network of brassinosteroid on seed shattering, the abscission zone of E. sibiricus cv. Lanyu No.
Huanhuan Lu, Yiyang Wan, Liuban Tang, Yongsen Qiu, Yancui Zhao, Yuying Zheng, Wengang Xie   +6 more
wiley   +1 more source

Ureteroscopic Removal of Left Ureteral Lithiasis in a Patient with Acid Maltase Deficiency Disease

, 1993
Acid maltase deficiency is a rare cause of respiratory insufficiency (1). Clinical and biochemical manifestations of the disease arise from a deficiency of the lysosomal enzyme α1,4−glucosidase (acid maltase) which degrades glycogen to free glucose (2 ...
Grogono, Alan, Garth, Kevin, Gitlin, Melvin, Jahr, Jonathan   +3 more
core   +1 more source