Results 101 to 110 of about 67,076 (344)

Metabolic Patterning on a Chip: Towards in vitro Liver Zonation of Primary Rat and Human Hepatocytes

, 2018
An important number of healthy and diseased tissues shows spatial variations in their metabolic capacities across the tissue. The liver is a prime example of such heterogeneity where the gradual changes in various metabolic activities across the liver ...
Eo, Jinsu, Kang, Young Bok (Abraham), Mert, Safak, Usta, O. Berk, Yarmush, Martin L.   +4 more
core   +1 more source

ADAR1 as a Placental Innate Immune Rheostat Sustaining the Homeostatic Balance of Intrinsic Interferon Response at the Maternal‐Fetal Interface

Advanced Science, EarlyView.
This study reveals that ADAR1, an RNA‐editing enzyme, fine‐tunes immune responses in the placenta by preventing the accumulation of immunogenic double‐stranded RNAs (dsRNAs) from interferon‐stimulated genes. The loss of ADAR1 in the placenta leads to excessive interferon signaling restricted to the junctional zone, disrupting placental development and ...
Xiaogang Chen, Xiangchao Xu, Jiahao Chen, Huiru Zhang, Wanshan Zheng, Wenzhe Yu, Xiaoqian Hu, Bin Cao   +7 more
wiley   +1 more source

Hypercalcemia in glycogen storage disease type I patients of Turkish origin

The Turkish Journal of Pediatrics, 2012
Glycogen storage disease type I (GSD I) is an autosomal recessive disorder caused by defects in the glucose-6-phosphatase complex. Deficient activity in the glucose-6-phosphatase-a (G6Pase) catalytic unit characterizes GSD IA and defects in the ...
Ciğdem Seher Kasapkara, Leyla Tümer, Ilyas Okur, Tuba Eminoğlu, Fatih Süheyl Ezgü, Alev Hasanoğlu   +5 more
doaj  

LncRNA Foxo6os as a Novel “ Scaffold” Mediates MYBPC3 in Combating Pathological Cardiac Hypertrophy and Heart Failure

Advanced Science, EarlyView.
Schematic overview showing that forkhead box O6, opposite strand (Foxo6os) acts as a “scaffold”, directly binding myosin‐binding protein‐C (MYBPC3) and recruiting protein kinase C (PKC‐α) to mediate site‐specific phosphorylation of MYBPC3. This post‐translational modification supports cardiac contraction by regulating L‐type Ca2+ channels, especially ...
Jie Sheng, Qin Lin, Yizhuo Sun, Yilei Meng, Sangyu Hu, Huaming Cao, Fang Lin, Yuping Zhu, Luying Peng, Li Li   +9 more
wiley   +1 more source

Over 20-Year Follow-up of Patients with Hepatic Glycogen Storage Diseases: Single-Center Experience

Diagnostics, 2020
Background: The published data on the long-term outcomes of glycogen storage disease (GSD) patients is sparse in the literature. The aim of this study was to analyze the long-term (over 20 years) follow-up of patients with hepatic types of GSD-I, III, VI,
Edyta Szymańska, Patryk Lipiński, Dariusz Rokicki, Janusz Książyk, Anna Tylki-Szymańska   +4 more
doaj   +1 more source

Retromer Regulates Macro‐ and Micro‐Autophagy via Distinct Vacuolar Proteases in the Rice Blast Fungus

Advanced Science, EarlyView.
In wild‐type of M. oryzae (Guy11), MoPrb1 and MoPep4 are delivered into the vacuole by the retromer complex to mediate protein degradation and selective autophagy. Their proper localization and catalytic functions are essential for the fungal vegetative growth, conidiation and pathogenicity. However, retromer mutations disrupt this transport, impairing
Dingyang Zhang, Jiexiong Hu, Yonghe Hong, Yuping Fan, Minghui Peng, Yakubu Saddeeq Abubakar, Shujing Liang, Nan Jiang, Lili Lin, Xiaohong Pan, Huakun Zheng, Naweed I. Naqvi, Zonghua Wang, Wenhui Zheng   +13 more
wiley   +1 more source

Glycogen Storage Disease Type Ib in the Compound Heterozygous State: Case Report

Педиатрическая фармакология
Background. Glycogen Storage Disease Type Ib (GSDIb) is an inherited autosomal recessive orphan disease associated with a deficiency of the glucose—6-phosphate translocase transport protein and leading to excessive accumulation of glycogen in the liver ...
Nikol P. Shimkova, Mariya A. Snegurenko, Eva Yunkevich, Nataliya V. Klimina, Anastasiya A. Shepina, Lyubov E. Larina   +5 more
doaj   +1 more source

Renal Disease in an Adult Patient with Type I Glycogen Storage Disease

Renal Failure, 1995
A 26-year-old Chinese male patient with type I glycogen storage disease presented with chronic renal disease, proteinuria, and urolithiasis. On renal biopsy, focal glomerular sclerosis, increased mesangial matrix and cellularity, interstitial fibrosis, tubular atrophy, and prominent arteriosclerosis were observed. Immunofluorescence microscopy revealed
Benny Kho, Paik-Seong Lim
openaire   +3 more sources

Modulation of Aging Diseases via RAGE Targets: A Dietary Intervention Review

Advanced Science, EarlyView.
This paper analyzes RAGE ligand‐binding mechanisms and signaling, proposing intervention strategies targeting these interactions. It emphasizes dietary polyphenols, polysaccharides, and terpenoids delivered via functional foods for anti‐aging. These compounds offer long‐term, safe intervention through dietary supplementation, providing dual nutritional
Qian Wu, Jia Yan, Yuyan Zhang, Xiaozhi Ming, Siqi Chen, Zhichao Zou, Nianjie Feng, Jianbo Xiao   +7 more
wiley   +1 more source

NRF2 signalling in cytoprotection and metabolism

British Journal of Pharmacology, EarlyView., 2023
The KEAP1‐NRF2 system plays a central role in cytoprotection in defence mechanisms against oxidative stress. The KEAP1‐NRF2 system has been regarded as a sulfur‐utilizing cytoprotective mechanism, because KEAP1 serves as a biosensor for electrophiles by using its reactive thiols and NRF2 is a transcriptional factor regulating genes involved in sulfur ...
Shohei Murakami, Yusuke Kusano, Keito Okazaki, Takaaki Akaike, Hozumi Motohashi   +4 more
wiley   +1 more source