Results 61 to 70 of about 6,177 (249)

Clinical phenotype and trio whole exome sequencing data from a patient with glycogen storage disease IV in Indonesia(NCBI)

Data in Brief
Glycogen storage disease type IV (GSD IV) is a rare disease caused by a defect in glycogen branching enzyme 1 (GBE1), which played a crucial role in glycogen branching.
Ivan William Harsono, Yulia Ariani, Beben Benyamin, Fadilah Fadilah, Dwi Ari Pujianto, Cut Nurul Hafifah, Titis Prawitasari   +6 more
doaj   +1 more source

Migrasomes in Ischemic Stroke: Molecular Landscape and Pathophysiological Impact

Advanced Science, EarlyView.
Multi‐omics profiling of stroke patients exposes systemic molecular fingerprints of immune activation, oxidative stress and metabolic collapse. Complement and coagulation cascade, and cholesterol metabolism emerge as shared functional hallmarks of plasma migrasomes in stroke patients and migrasomes isolated from experimental stroke mice.
Huifen Zhou, Yingying Zhang, Peng Zhou, Haofang Wan, Lingling Li, Bin Xu, Yuping Wan, Yingming Song, Yi Kang, Hongbo Zhang, Min Shi, Qun Hou, Jiehong Yang, Chen Ding, Wei Fu, Buchang Zhao, Haitong Wan   +16 more
wiley   +1 more source

Living Donor Liver Transplantation in a Korean Child with Glycogen Storage Disease Type IV and a GBE1 Mutation

Gut and Liver, 2009
Glycogen storage disease type IV (GSD-IV) is an autosomal recessive disease caused by a deficient glycogen branching enzyme (GBE), encoded by the GBE1 gene, resulting in the accumulation of abnormal glycogen deposits in the liver and other tissues.
doaj   +1 more source

Structural features of the glycogen branching enzyme encoding genes from aspergilli

Microbiological Research, 2002
A maltose binding protein, p78, was purified to homogeneity from Aspergillus nidulans by a single column chromatography step on cross-linked amylose. The partial amino acid sequence was highly homologous to the glycogen branching enzymes (GBEs) of human and yeast, and p78 did show branching enzyme activity.
Sasangka, Prasetyawan, Matsuno, Aya, Tanaka, Akimitsu, Akasaka, Yuki, Suyama, Sachie, Kano, Sumie, Miyazaki, Makiko, Akao, Takeshi, Kato, Masashi, Kobayashi, Tetsuo, Tsukagoshi, Norihiro   +10 more
openaire   +2 more sources

Deciphering the catalytic and pharmacological mechanisms of Coptis chinensis herbzymes to renovate intestinal microenvironment for colitis alleviation

BMEMat, EarlyView.
The synthesized CCzymes possess both antioxidant enzyme activity and pharmacological properties inherent to Coptis chinensis. By their antioxidant enzyme activity, CCzymes can attenuate oxidative stress within the inflammatory region of ulcerative colitis (UC), while their pharmacological activity acts on macrophage polarization and the intestinal ...
Zhichao Deng, Can Li, Yujie Zhang, Yuanyuan Zhu, Chenxi Xu, Bowen Gao, Mingxin Zhang, Martina H. Stenzel, Kelong Fan, Mingzhen Zhang, Yanyan Jiang   +10 more
wiley   +1 more source

The myth of the metabolic baseline: sleep–wake cycles undermine a foundational assumption in organismal biology

Biological Reviews, EarlyView.
ABSTRACT Basal and standard metabolic rate (BMR and SMR) are cornerstones of physiological ecology and are assumed to be relatively fixed intrinsic properties of organisms that represent the minimum energy required to sustain life. However, this assumption is conceptually flawed. Many core maintenance processes underlying SMR are temporally partitioned
Helena Norman, Daphne Cortese, Amelia Munson, Jan Lindström, Shaun S. Killen   +4 more
wiley   +1 more source

Systemic aging fuels heart failure: Molecular mechanisms and therapeutic avenues

ESC Heart Failure, Volume 12, Issue 2, Page 1059-1080, April 2025.
Abstract Systemic aging influences various physiological processes and contributes to structural and functional decline in cardiac tissue. These alterations include an increased incidence of left ventricular hypertrophy, a decline in left ventricular diastolic function, left atrial dilation, atrial fibrillation, myocardial fibrosis and cardiac ...
Zhuyubing Fang, Umar Raza, Jia Song, Junyan Lu, Shun Yao, Xiaohong Liu, Wei Zhang, Shujuan Li   +7 more
wiley   +1 more source

Inherited metabolic epilepsies–established diseases, new approaches

Epilepsia Open, EarlyView.
Abstract Inherited metabolic epilepsies (IMEs) represent the inherited metabolic disorders (IMDs) in which epilepsy is a prevailing component, often determining other neurodevelopmental outcomes associated with the disorder. The different metabolic pathways affected by individual IMEs are the basis of their rarity and heterogeneity.
Itay Tokatly Latzer, Phillip L. Pearl
wiley   +1 more source

A novel genetic model provides a unique perspective on the relationship between postexercise glycogen concentration and increases in the abundance of key metabolic proteins after acute exercise.

PLoS ONE
Some acute exercise effects are influenced by postexercise (PEX) diet, and these diet-effects are attributed to differential glycogen resynthesis. However, this idea is challenging to test rigorously. Therefore, we devised a novel genetic model to modify
Seong Eun Kwak, Amy Zheng, Edward B Arias, Haiyan Wang, Xiufang Pan, Yongping Yue, Dongsheng Duan, Gregory D Cartee   +7 more
doaj   +1 more source

Harnessing Piezoelectric Biomaterials for Pathogenic Eradication and Tissue Regeneration

Exploration, EarlyView.
This review highlights recent advances in piezoelectric biomaterials for antibacterial therapy and tissue regeneration, discussing material classifications, catalytic mechanisms, property enhancements, biomedical applications, and future challenges in clinical translation.
Wenxuan Mao, Xiaoye Li, Ao He, Meng Ding, Yu Zhang, Zhuo Dai, Qiang Li, Weijun Xiu, Yanling Hu, Yongbin Mou, Dongliang Yang, Heng Dong   +11 more
wiley   +1 more source