Results 71 to 80 of about 526,926 (248)

Frequency of Fabry disease in a juvenile idiopathic arthritis cohort

Pediatric Rheumatology Online Journal, 2021
Background Fabry disease (FD) is a rare, X-linked, multisystemic lysosomal storage disorder (LSD) that results from a deficiency in the hydrolase alpha-galactosidase A (⍺-GalA). During childhood, classic FD symptomatology is rare.
Luciana Paim-Marques, Amanda Virginia Cavalcante, Islane Verçosa, Paula Carneiro, Marcia Souto-Maior, Erlane Marques, Simone Appenzeller   +6 more
doaj   +1 more source

Senescent Cell Derived Artificial Vesicle‐Based Senolytic Sonovaccine Platform with Augmented Lymph Node Delivery and Antigen Cross‐Presentation Efficacy

Advanced Science, EarlyView.
A senolytic sonovaccine platform (SenoVac) is developed, in which senescent cell‐derived vesicles serve as broad senescent cell antigen reservior. The “2‐step” click chemistry strategy for effective lymph node delivery, and ultrasound‐triggered endosomal escape to boost cross‐presentation, ensures efficient senescent cell clearance and disease ...
Liang Zhang, Yubo Lai, Jia Wang, Lingling Liu, Jieyuan An, Mi Qu, Yuan Liang, Bijun Tan, Guodong Yang, Xuekang Yang, Lijun Yuan   +10 more
wiley   +1 more source

Fabry Disease - literature review

Journal of Education, Health and Sport
Introduction and objective: Fabry disease (FD) is a rare lysosomal storage disorder that can manifest in classical and atypical forms, with the latter being more common. It results from deficient alpha-galactosidase activity, leading to the accumulation
Krystian Wdowiak, Agnieszka Maciocha, Julia Wąż, Aleksandra Witas, Adrian Muzyka, Julia Rydzek, Ewa Gardocka   +6 more
doaj   +1 more source

Activity of α-d-Galactosidase in Long-Stored Seeds of Vicia hirsuta

, 2023
Mature seeds of many crop species contain substantial amounts of soluble carbohydrates, including raffinose family oligosaccharides (RFOs) and cyclitol galactosides (Gal-C).
Ewa Gojło
core   +1 more source

SIRT6‐Mediated Deacetylation of ATF3 Promotes Silica‐Induced Lung Fibrosis by Enhancing its Nuclear Import via Binding to Importin α

Advanced Science, EarlyView.
SIRT6‐mediated ATF3 acetylation drives MGARP transcription and mitochondrial dysfunction in macrophages, promoting macrophage senescence and pulmonary fibrosis. Mechanistically, HSP70/Importin α competitively binds to ATF3, modulating its nuclear translocation.
Demin Cheng, Wenxia Bu, Fengxu Wang, Yueyuan Jin, Rongzhu Liu, Rui Zhao, Xuehai Wang, Mengna Jiang, Jinping Shen, Xinhang Cheng, Zuming Chen, Li Zhu, Jinlong Li, Zhenzhong Ge, Shichen Miao, Haotian Xu, Xiaoyu Zhou, Dongming Wang, Xinyuan Zhao   +18 more
wiley   +1 more source

Removal of flatulence causing sugars in soymilk

, 2009
The enzyme, alpha-galactosidase was extracted from peanuts using various buffers and solvents. The specific activity of soluble preparation of peanut ranged from 0.0083 to 0.0606 U/mg.
Rajan, Anila
core  

Renal IGFBP6 Interacts With THBS1 to Drive Renal Cellular Senescence and Fibrosis

Advanced Science, EarlyView.
ABSTRACT Epithelial dedifferentiation and myofibroblast activation are critical drivers of chronic kidney disease (CKD) progression. Elevated levels of IGFBP6 have been linked to decreased renal function in CKD patients, but its precise role and underlying mechanisms remain unclear.
Ju‐tao Yu, Chang‐geng Xu, Xiao‐wei Hu, Run‐run Shan, Jia‐nan Wang, Xiang‐yu Li, Yu‐qing Wang, Yu‐hang Dong, Shen‐Zhuo‐Fan Huang, Shuai Sun, Tian Zhao, Ya‐Xin Ru, Xiao‐yu Shen, Rui Hou, Jian‐min You, Fei Zhang, Chao Li, Jie Wei, Chao Hou, Zhi‐juan Wang, Dan‐feng Zhang, Juan Jin, Jia‐gen Wen, Yu‐jie Liu, Ming‐ming Liu, Xiao‐ming Meng, Wei Wang   +26 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

Data_Sheet_1_Bioconversion of Beet Molasses to Alpha-Galactosidase and Ethanol.docx

, 2019
Molasses are sub-products of the sugar industry, rich in sucrose and containing other sugars like raffinose, glucose, and fructose. Alpha-galactosidases (EC.
María-Esperanza Cerdán (5712044), María-Isabel González-Siso (5712050), María-Efigenia Álvarez-Cao (5712038), Manuel Becerra (5712047)   +3 more
core   +1 more source

Cerebral hemorrhage in Fabry's disease [PDF]

, 2010
Fabry's disease is an X-linked lysosomal storage disorder resulting from alpha-galactosidase A deficiency. Although ischemic stroke is recognized as an important manifestation of Fabry's disease, hemorrhagic stroke is considered to be rare.
Sekijima, Yoshiki, Shimizu, Yusaku, Katsuya Nakamura, Yoshimitsu Fukushima, Hattori, Kiyoko, Endo, Fumio, Fumio Endo, Ikeda, Shu-ichi, Kimitoshi Nakamura, Takuji Yasude, Masao Ushiyama, Nakamura, Kimitoshi, Kiyoshiro Nagamatsu, Ushiyama, Masao, Yusaku Shimizu, Nakamura, Katsuya, Kiyoko Hattori, Nagamatsu, Kiyoshiro, Yoshiki Sekijima, Fukushima, Yoshimitsu, Shu-ichi Ikeda   +20 more
core   +1 more source