Results 41 to 50 of about 900,860 (290)

TRAIP Mediates Alcohol‐Induced Liver Injury through Regulating β‐catenin Ubiquitin Degradation via Direct Interaction

Advanced Science, EarlyView.
This study reveals that NF‐κB1‐driven TRAIP upregulation in ALD correlates with disease severity. Mechanistically, TRAIP directly binds β‐catenin via its CC domain and promotes its K48‐linked ubiquitination and degradation, which is independent of the GSK3β/β‐TrCP pathway.
Zhan Wu, Mingjiang Liu, Lijuan Liao, Zhaohong Mo, Wenli Xu, Linfeng Mao, Yubing Chen, Qiuli Xie, Ming Yao, Shilian Chen, Stephen Tomlinson, Sebastian Mueller, Songqing He, Guandou Yuan   +13 more
wiley   +1 more source

Prevalence of Anderson-Fabry disease in male patients with late onset hypertrophic cardiomyopathy [PDF]

, 2002
Background-Although studies have suggested that "late-onset" hypertrophic cardiomyopathy (HCM) may be caused by sarcomeric protein gene mutations, the cause of HCM in the majority of patients is unknown.
Tei, C, B. Sachdev, Takenaka, T, W. J. Mckenna, Sachdev, B, H. Teraguchi, Elliott, PM, Teraguchi, H, C. Tei, P. Lee, T. Takenaka, McKenna, WJ, Lee, P, Hypertrophic Cardiomyopathy   +13 more
core  

Ultrasound‐Activatable Piezoelectric Hydrogel Reprograms Mitochondrial Epigenetics for Osteoarthritis Therapy via the mTOR/GATD3A Axis

Advanced Science, EarlyView.
An ultrasound‐activatable piezoelectric hydrogel reprograms chondrocyte mitochondrial epigenetics via the mTOR/GATD3A axis, clearing damaged mitochondria and alleviating osteoarthritis progression in both mouse models and human cartilage explants. ABSTRACT The avascular nature of cartilage hinders drug delivery for osteoarthritis (OA) therapy.
Hui Zheng, Pengfei Yan, Peng Liu, Chang Yan, Mengqi Zhao, Zuyong Wang, Chao Ma, Rongkai Zhang, Xi Cui, Swee Hin Teoh   +9 more
wiley   +1 more source

When should we think about Fabry disease?

Journal of Education, Health and Sport
According to the European Union definition, a rare disease is a disease that occurs with a frequency of less than 5 per 10,000. Rare diseases pose a major diagnostic problem for physicians.
Natalia Chojnacka, Radosław Cymer, Karolina Jurasz, Dominika Podgórska, Ewa Rzeska, Miłosz Sanecki, Karolina Tomczyk, Jakub Klarycki   +7 more
doaj   +1 more source

Glycosidases of ehrlich ascites tumor cells and ascitic fluid--Purification and substrate specificity of [alpha]-N-Acetylgalactosaminidase and [alpha]-galactosidase: Comparison with coffee bean [alpha]-galactosidase

, 1990
Ehrlich ascites tumor cells and ascitic fluid were assayed for glycosidase activity. [alpha]-Galactosidase and [beta]-galactosidase, [alpha]- and [beta]-mannosidase, [alpha]-N-acetylgalactosaminidase, and [beta]-N-acetylglucosaminidase activities were ...
Goldstein, Irwin J., Eckhardt, Allen E., Yagi, Fumio   +2 more
core   +1 more source

Human Urine Stem Cells Alleviate Pulmonary Fibrosis via Inhibiting Macrophage‐Myofibroblast Transition

Advanced Science, EarlyView.
Therapeutic role and mechanism of human urine stem cells (hUSCs) in pulmonary fibrosis. hUSCs alleviated pulmonary fibrosis by selectively inhibiting macrophage‐myofibroblast transition (MMT) in two ways: on one hand, hUSCs inhibited mitochondrial reactive oxygen species (mtROS) production and apoptosis/senescence of epithelial cells in pulmonary ...
Zhou‐Hang Zhang, Guan‐Lin Guo, Xiao‐Hui Guan, Min Hu, Qi‐Ming Huang, Ding‐Wen Guo, Hao‐Cheng Gu, You‐Qiong Zhuo, Ning Li, Hong‐Bo Xin, Ke‐Yu Deng   +10 more
wiley   +1 more source

Ultrastructural deposits appearing as “zebra bodies” in renal biopsy: Fabry disease?– comparative case reports

BMC Nephrology, 2017
Background Fabry Disease (FD) is a genetic disorder caused by alpha-galactosidase A deficiency. Certain drugs, such as hydroxychloroquine, can produce renal deposits that mimic morphological findings seen in FD, characterizing a type of drug-induced ...
Precil Diego Miranda de Menezes Neves, Juliana Reis Machado, Fabiano Bichuette Custódio, Maria Luíza Gonçalves dos Reis Monteiro, Shigueo Iwamoto, Marlene Freire, Marisa França Ferreira, Marlene Antônia dos Reis   +7 more
doaj   +1 more source

Targeting NSUN2‐Mediated m5C Modification Attenuates Chondrocyte Senescence and NLRP3 Activation in Osteoarthritis

Advanced Science, EarlyView.
NSUN2‐mediated m5C modification cooperates with ALYREF to stabilize and export IP3R3 mRNA, increasing IP3R3 expression and Ca2 + overload in chondrocytes. This signaling promotes mitochondrial dysfunction, NLRP3 inflammasome activation, and senescence, thereby accelerating osteoarthritis progression.
Guping Mao, Wei Li, Zhencan Lin, Zengfa Deng, Ming Li, Zongrui Jiang, Changzhao Li, Dianbo Long, Yan Kang   +8 more
wiley   +1 more source

Cell Transplantation Combined with Recombinant Collagen Peptides for the Treatment of Fabry Disease

Cell Transplantation, 2020
Fabry disease is caused by a decrease in or loss of the activity of alpha-galactosidase, which causes its substrates globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3) to accumulate in cells throughout the body. This accumulation results
Daisuke Kami, Masashi Yamanami, Takahiro Tsukimura, Hideki Maeda, Tadayasu Togawa, Hitoshi Sakuraba, Satoshi Gojo   +6 more
doaj   +1 more source

HMGCR‐Driven Cholesterol Metabolism Promotes Osteoarthritis Progression by Accelerating Synovial Fibroblast Senescence

Advanced Science, EarlyView.
In the pathological context of osteoarthritis (OA), the phosphorylation of AKT1 at Ser473 enhances its binding to Lys140 of Insig1, which facilitates the formation of AKT1–Insig1 complex. Subsequently, the activation of AKT1 promotes the phosphorylation of Insig1 at Ser189, potentially enhancing the dissociation of Insig1 from sterol regulatory element‑
Xiaoqi Zhang, Siru Zhou, Xi Liu, Wei Xiang, Xiuqin Peng, Yu Tian, Song Li, Hailin Wang, Bo Liao, Shunzheng Fang, Runze Jin, Jinhui Wu, Chuanqing Bai, Wei Zhang, Bo Huang, Bingfei Li, Lifeng Chen, Lin Chen, Bin Zhang, Zhenhong Ni   +19 more
wiley   +1 more source