Results 51 to 60 of about 31,724,613 (234)

Modifiable factors affecting renal preservation in type I glycogen storage disease after liver transplantation: a single-center propensity-match cohort study

Orphanet Journal of Rare Diseases, 2021
Background and aims Glycogen storage disease type I (GSD-I) is an autosomal recessive disorder of carbohydrate metabolism, resulting in limited production of glucose and excessive glycogen storage in the liver and kidneys.
Yi-Chia Chan, Kai-Min Liu, Chao-Long Chen, Aldwin D. Ong, Chih-Che Lin, Chee-Chien Yong, Pei-Chun Tsai, Liang-Suei Lu, Jer-Yuarn Wu   +8 more
doaj   +1 more source

SPARC Drives Podocyte Mitochondrial Damage and Ferroptosis in Diabetic Kidney Disease Following Klotho Deficiency

Advanced Science, EarlyView.
Klotho deficiency promotes podocyte mitochondrial dysfunction and ferroptosis through activation of the PKCα/CUX1/SPARC/TGFβ‐RII axis. SPARC emerges as a key mediator linking Klotho loss to podocyte injury in DKD and other kidney injury models, suggesting broader implications for CKD progression.
Qing Yang, Huimin Jiang, Chenyang Xiong, Xindi Zhou, Qinqin Tan, Han Xiao, Lan Qiu, Chun Gan, Yaxi Chen, Haiping Yang, Wei Jiang, Qiu Li   +11 more
wiley   +1 more source

Chemotherapy‐Activated GSK3β‐DNMT1 Signaling Upregulates CD47 to Evade Macrophage Phagocytosis and Drive Temozolomide Resistance in Glioblastoma

Advanced Science, EarlyView.
Temozolomide treatment activates GSK3β, driving DNMT1 phosphorylation, destabilization, and CD47 promoter hypomethylation in glioblastoma. This epigenetic shift upregulates CD47, enabling TMZ‐treated GBM cells to evade macrophage phagocytosis, survive chemotherapy, and acquire resistance.
Jie Li, Shuai Ying, Yi Zhang, Jingjing Wang, Quan Wan, Lingli Gong, Daxing Xu, Zhenkun Yang, Hongnian Lu, Ying Yin, Xiangming Fang, Zhening Pu   +11 more
wiley   +1 more source

Newborn Screening for Pompe Disease

International Journal of Neonatal Screening, 2020
Glycogen storage disease type II (also known as Pompe disease (PD)) is an autosomal recessive disorder caused by defects in α-glucosidase (AαGlu), resulting in lysosomal glycogen accumulation in skeletal and heart muscles.
Takaaki Sawada, Jun Kido, Kimitoshi Nakamura   +2 more
doaj   +1 more source

Self‐Assembling Peptide‐Adjuvant Conjugate (SaPAC) Platform for Precision Cancer Immunotherapy

Advanced Science, EarlyView.
A self‐assembling peptide–adjuvant conjugate links neoantigens to TLR7 agonism, forming cationic nanoparticles that enhance lymph‐node retention, dendritic‐cell activation, and antitumor T‐cell immunity. This chemically defined platform shows how synchronized antigen and innate stimulation can be adapted across melanoma, bladder cancer, and triple ...
Yang‐Fan Wu, Jing‐Chu Hu, Ye‐Fan Hu, Wen‐Jun Li, Li Rong, Ren‐Hao Li, Yuan Yao, Lehan Hu, Xiao‐Lei Wang, Bao‐Zhong Zhang, Yicheng Lu, Shing‐Fung Chow, Canhui Su, Thomas Yau, Clive Yik‐Sham Chung, Jian‐Dong Huang   +15 more
wiley   +1 more source

Alternative nighttime nutrition regimens in glycogen storage disease type I: a controlled crossover study [PDF]

, 2015
BACKGROUND Traditional approaches for nighttime glycemic control in glycogen storage disease type I (GSDI) include continuous tube feeding, or ingestion of uncooked corn starch (CS) at bedtime.
Krützfeldt, Jan, Baumgartner, Matthias R, Roger Lehmann, Hochuli, Michel, Matthias R. Baumgartner, Michel Hochuli, Lehmann, Roger, Emanuel Christ, Christ, Emanuel, Meienberg, Fabian, Jan Krützfeldt, Fabian Meienberg   +11 more
core   +1 more source

Decoding Biomolecular Phase Separation: From Physiological Roles to Pathological Mechanisms and Therapeutic Biomaterials Design

Advanced Science, EarlyView.
This review systematically elaborates on the mechanisms underlying physiological regulation and pathological dysregulation of biomolecular phase separation. It also summarizes advances in phase‑separation‑targeted therapeutic strategies and the design of functional biomaterials inspired by this process.
Jiawei Zhu, Jingbo Dong, Qiufang Gong, Xuejiao Song, Zhifa Shen, Chao Liang   +5 more
wiley   +1 more source

Glucose-6 Phosphate, A Central Hub for Liver Carbohydrate Metabolism

Metabolites, 2019
: Cells efficiently adjust their metabolism according to the abundance of nutrients and energy. The ability to switch cellular metabolism between anabolic and catabolic processes is critical for cell growth.
Fabienne Rajas, Amandine Gautier-Stein, Gilles Mithieux   +2 more
doaj   +1 more source

Excessive production of uric acid in type I glycogen storage disease

, 1968
An increased de novo production of uric acid has been demonstrated in three hyperuricemic siblings with Type I glycogen storage disease. The hyperuricemia in Type I glycogen storage disease, therefore, appears to result from the previously demonstrated ...
Seegmiller, J.E, Howell, R. Rodney, W.N. Kelley, Kelley, W.N, F.M. Rosenbloom, J.E. Seegmiller, Rosenbloom, F.M, R. Rodney Howell   +7 more
core   +1 more source

A Postbiotic‐Inducing Ganoderma applanatum Polysaccharide Repairs the Intestinal Barrier via a Phocaeicola vulgatus ‐ Tridecylic Acid Axis

Advanced Science, EarlyView.
This study identifies GAP2, a bioactive polysaccharide fraction derived from Ganoderma applanatum, as a postbiotic inducer that repairs intestinal barrier damage in a gut microbiota‐dependent manner. GAP2 enriches Phocaeicola vulgatus, which degrades GAP2 and promotes tridecylic acid production, triggering Rho/CDC42 signaling and tight‐junction ...
Chuan Zhang, Miaoyu Li, Tiantian Li, Xiaojie Zang, Haiyang Shi, Yaru Liu, Chengcheng Zhang, Jianxin Zhao, Qixiao Zhai, Wei Chen, Fengwei Tian   +10 more
wiley   +1 more source