Results 71 to 80 of about 118,748,616 (241)

alpha-Galactosidase delivery using 30Kc19-human serum albumin nanoparticles for effective treatment of Fabry disease

, 2016
Fabry disease is a genetic lysosomal storage disease caused by deficiency of alpha-galactosidase, the enzyme-degrading neutral glycosphingolipid that is transported to lysosome.
Park, Hee Ho, Sohn, Youngsoo, Park, Tai Hyun, Ryu, Jina, Rhee, Won Jong, Park, Ju Hyun, Lee, Hong Jai   +6 more
core   +1 more source

Purification, characterization, and partial nucleotide sequencing of soybean alpha-galactosidase

, 1991
Soybeans (Glycine max) contain an $\alpha$-galactosidase that makes up 0.05% of the total protein of the seed. $\alpha$-Galactosidase hydrolyzes stachyose and raffinose into galactose and sucrose, the initial energy sources in the germinating seed.
Porter, Jill Elizabeth
core   +1 more source

Restores Aβ Clearance by Overcoming PCSK9–LRP1 Dysregulation and TRIB3‐Mediated Autophagy Blockade in Alzheimer's Disease

Advanced Science, EarlyView.
PCSK9 upregulation in cerebrovascular endothelial cells and microglia destabilizes the Aβ transporter LRP1, while TRIB3‐associated autophagy blockade further limits intracellular Aβ degradation. A RAP‐modified tea‐polyphenol nanoparticle co‐delivers siPCSK9 and siTRIB3 to restore LRP1‐mediated Aβ efflux and autophagy‐dependent degradation, reducing Aβ ...
Jie Miao, Jing Wang, Yankun Li, Haipei Zhang, Yanli Zhang, Qingnian Li, Bo Xiao, Wenhu Zhou, Junhong Guo   +8 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

Topological Quantum Biomaterials Enable Pyroptosis‐Senescence Coupling for Enhanced Colorectal Cancer Therapy

Advanced Science, EarlyView.
This work reports conceptually unprecedented topological quantum biomaterials derived from engineered Pd3Sn nanostrips for oncological applications. Their near‐Fermi‐level electronic architecture and superior interfacial charge transport facilitate ultrasound‐amplified redox reactions and efficient ROS generation.
Zilu Chen, Kun Mei, Yan Yang, Mengna Xu, Xinyue Dai, Renjun Gu, Fuqiong Zhou, Cheng Wang, Meiqi Chang, Yan Huang, Yu Chen   +10 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

Yoda1‐Functionalized Nanozymes Coordinate Piezo1 Activation and Mitochondrial Protection to Reprogram the Diabetic Osteoimmune Micromilieu and Promote Vascularized Bone Repair

Advanced Science, EarlyView.
Diabetic bone defects remain a critical clinical challenge. A 3D‐printed BCPG‐MF@Y scaffold, embedding Yoda1‐functionalized manganese dioxide‐ferritin nanozymes, scavenges mtROS, suppresses the cGAS–STING pathway, and reverses cellular senescence, while Yoda1 activates Piezo1 to direct BMSCs fate to synergistically reprogram the osteoimmune ...
Qing‐Song Deng, Ang Wang, Ren Zhang, Xu‐Ran Li, Po‐Lin Liu, Shu‐Hang He, Zi‐Yin Han, Zhi‐Han Shen, Zhan‐Ying Wei, Chang‐Ru Zhang, Fei Wang, Helen Dawes, Jian Lu, Tong‐He Zhu, Jin‐Zhong Zhao, Shang‐Chun Guo, Shi‐Cong Tao   +16 more
wiley   +1 more source

Determination of Gene‐Level Fitness Contributions Across the Mucin‐Utilization Network of Bacteroides thetaiotaomicron

Advanced Science, EarlyView.
Genome‐wide CRISPRi and RNA‐seq resolve how B. thetaiotaomicron converts host mucosal glycans into fitness. The study reveals a layered mucin‐utilization architecture in which capsular polysaccharides constrain surface access, extracellular CAZymes and peptidases show functional redundancy, and intracellular catabolic pathways create discrete metabolic
Kangsan Kim, Jiwon Lee, Minjeong Kang, Sung Sun Yim, Donghui Choe, Byung‐Kwan Cho   +5 more
wiley   +1 more source

Production of secreted guar alpha-galactosidase by Lactococcus lactis

, 1995
A plant alpha-galactosidase gene was inserted in the expression vector pGKV259. The resulting plasmid pGAL2 consisted of the replication functions of the broad-host-range lactococcal plasmid pWVO1, the lactococcal promoter P59, and the DNA sequences ...
Bolhuis, A, Leenhouts, KJ, Kok, J, Ledeboer, A, Venema, G   +4 more
core   +1 more source

Identification and Clinical Characterization of a Novel Alpha-Galactosidase A Mutation

Journal of Inborn Errors of Metabolism and Screening, 2014
Fabry disease (FD) is an inborn error of metabolism characterized by deficient/absent activity of lysosomal enzyme alpha-galactosidase A, which results in systemic accumulation of glycosphingolipids and progression to renal failure, heart and ...
Nilton Salles Rosa Neto MD, PhD
doaj   +1 more source