Results 81 to 90 of about 130,693 (343)

A Brain‐Penetrant Nanobody Reveals GSK3β‐Driven Proline‐Directed Phosphorylation as a Master Regulator of Ischemic Neurodegeneration

Advanced Science, EarlyView.
A brain‐targeted nanoparticle enables delivery of a therapeutic nanobody (Nb.29E9) that inhibits pathogenic GSK3β signaling. This intervention restores AMPK/mTORC1/TGFβ homeostasis, attenuates neuroinflammation and oxidative stress, and promotes long‐term functional recovery after ischemic stroke.
Lan Li, Muyang Li, Lei Sun, Ying Yang, Yuanshun Wu, Ziyi Yin, Anni Wang, Peiyang Zhou, Shaoxiang Luo, Jian Chen, Jun Qin, Zhibing Ai, Zilong Yuan, Zhiqiang Dong, Min Zhang   +14 more
wiley   +1 more source

Mutation analysis of in a patient with glycogen storage disease-type Ib

Journal of International Medical Research, 2019
Objective The aim of the study was to investigate the relationship between SLC37A4 gene mutation and clinical phenotype in a patient with glycogen storage disease-type I.
Yamei Zhang, Huihui Sun, Naijun Wan
doaj   +1 more source

Fenofibrate rapidly decreases hepatic lipid and glycogen storage in neonatal mice with glycogen storage disease type Ia.

Human Molecular Genetics, 2019
Glycogen Storage Disease Type Ia (GSD Ia) is caused by autosomal mutations in G6PC and can present with severe hypoglycemia, lactic acidosis, and hypertriglyceridemia.
Zollie Yavarow, H. Kang, Lauren R. Waskowicz, B. Bay, S. Young, P. Yen, D. Koeberl   +6 more
semanticscholar   +1 more source

A Single‐Cell Transcriptomic Atlas of the Ovine Rumen Microbiome Characterizes Lineage‐Specific Metabolic Shifts Associated with Host Heat Tolerance

Advanced Science, EarlyView.
An optimized single‐cell transcriptomic framework profiles over 60 000 cells to map the ovine rumen microbiome, partitioning the ecosystem into seven cross‐species functional clusters. In heat‐resistant hosts, a lineage‐specific metabolic shift in Anaerovibrio lipolyticus toward a highly glycolytic phenotype contributes to a “nutritional sparing ...
Sanbao Zhang, Qinyang Jiang, Junjie Ma, Jianwei Zou, Fan Wang, Feifei Lv, Yanna Huang, Yongcheng Wang, Ziye Xu   +8 more
wiley   +1 more source

Pituitary hypoplasia and growth hormone deficiency in a woman with glycogen storage disease type Ia: a case report

Journal of Medical Case Reports, 2008
Introduction Growth retardation is one of the cardinal manifestations of glycogen storage disease type Ia. It is unclear which component of the growth hormone and/or insulin-like growth factor axis is primarily disrupted, and management of growth ...
Dagdelen Selcuk, Atmaca Aysegul, Alikasifoglu Ayfer, Erbas Tomris   +3 more
doaj   +1 more source

Downregulation of SIRT1 signaling underlies hepatic autophagy impairment in glycogen storage disease type Ia

PLoS Genetics, 2017
A deficiency in glucose-6-phosphatase-α (G6Pase-α) in glycogen storage disease type Ia (GSD-Ia) leads to impaired glucose homeostasis and metabolic manifestations including hepatomegaly caused by increased glycogen and neutral fat accumulation.
Jun-Ho Cho, Goo-Young Kim, C. Pan, J. Anduaga, E. Choi, B. Mansfield, J. Chou   +6 more
semanticscholar   +1 more source

Glycemic control and complications in glycogen storage disease type I: Results from the Swiss registry.

, 2019
BACKGROUND Regular carbohydrate intake to avoid hypoglycemia is the mainstay of dietary treatment in glycogen storage disease type I (GSDI). The aim of this study was to evaluate the quality of dietary treatment and glycemic control in a cohort of ...
Kaiser, Nathalie, Baumgartner, Matthias R, Hochuli, Michel, Bosanska, Lenka, Meienberg, Fabian, Spinas, Giatgen A, Gautschi, Matthias   +6 more
core   +1 more source

Characterization of the extracellular matrix from human and dog umbilical cords

The Anatomical Record, EarlyView.
Abstract The extracellular matrix is important for maintaining tissue morphogenesis and homeostasis; it can also be used as a biomaterial for the production of biological scaffolds. Particularly, the umbilical cord has shown potential in the production of scaffolds for small‐diameter vessels.
Ana Carla Mendonça, Roberta Losi Guembarovski, Carlos Alberto Miqueloto, Alda Fiorina Maria Losi Guembarovski, Caio Garcia Barbosa da Silva, Laís Capelasso Lucas Pinheiro, Phelipe Oliveira Favaron   +6 more
wiley   +1 more source

Prevention of complications in glycogen storage disease type Ia with optimization of metabolic control

Pediatric Diabetes, 2017
Prior to 1971, type Ia glycogen storage disease was marked by life‐threatening hypoglycemia, lactic acidosis, severe failure to thrive, and developmental delay.
Monika Dambska, E. Labrador, C. Kuo, D. Weinstein   +3 more
semanticscholar   +1 more source

Developmental Characteristics of the Embryonic Liver Tissue and Long‐Term Culture of Primary Hepatocytes in Duck

Animal Research and One Health, EarlyView.
Liver development was investigated at different embryonic ages (EAs). Furthermore, numerous hepatocyte media were formulated and evaluated. These results could elaborate the developmental characteristics of duck liver tissue and determine the most suitable medium for the proliferation and characteristic maintenance of hepatocytes in vitro, which would ...
Jie Wei, Jing Tang, Bo Zhang, Chenchen Du, Yunsheng Zhang, Ming Xie, Zhengkui Zhou, Shuisheng Hou   +7 more
wiley   +1 more source