Results 101 to 110 of about 78,667 (269)

Globotriaosylsphingosine Accumulation and Not Alpha-Galactosidase-A Deficiency Causes Endothelial Dysfunction in Fabry Disease

PLoS ONE, 2012
Fabry disease (FD) is caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (GLA) resulting in the accumulation of globotriaosylsphingosine (Gb3) in a variety of tissues. While GLA deficiency was always considered as the fulcrum of the disease, recent attention shifted towards studying the mechanisms through which Gb3 accumulation in ...
Namdar Mehdi, Gebhard Catherine, Studiger Rafael, Shi Yi, Mocharla Pavani, Schmied Christian, Brugada Pedro, Lüscher Thomas F, Camici Giovanni G   +8 more
openaire   +5 more sources

Precise Construction of an Antimicrobial Peptide Targeting Bacterial Cell Membranes Derived From Natural Peptides

Advanced Science, EarlyView.
An AMP (P 3‐3R‐8I) based on natural peptides, which can target bacterial cell membranes, was precisely constructed via amino acid mutation. P 3‐3R‐8I exhibits antibacterial capability which could be attributed to the ability of P 3‐3R‐8I to quickly penetrate bacterial cell membranes and then to bind to bacterial DNA.
Jiaqi Huang, Bohao Liu, Xingzhuo Zhu, Deqian Qiao, Sizhe Chen, Xiaoyan Zeng, Qingqing Yang, Zihuan Wei, Yinjuan Huang, Jizhao Wang, Guangjian Zhang, Qiuyu Gong   +11 more
wiley   +1 more source

Cardiac involvement in Anderson–Fabry disease. The role of advanced echocardiography

Frontiers in Cardiovascular Medicine
Anderson–Fabry disease (AFD) is a lysosomal storage disorder, depending on defects in alpha galactosidase A activity, due to a mutation in the galactosidase alpha gene. Cardiovascular involvement represents the leading cause of death in AFD.
Letizia Spinelli, Antonio Bianco, Eleonora Riccio, Antonio Pisani, Guido Iaccarino, Guido Iaccarino   +5 more
doaj   +1 more source

Photothermal–Immunomodulatory Hydrogel Reinforced by Ti3C2/ZnAl‐LDH Nanoplatform for Eradicating MRSA and Promoting Diabetic Wound Healing

Advanced Science, EarlyView.
A NETs‐inspired Ti3C2/LDH hydrogel integrates trapping, ROS boosting, and M2‐immunomodulation for MRSA‐infected wound repair. ABSTRACT MRSA infections give rise to chronic cutaneous and advance into profound tissue invasions, including osteomyelitis and sepsis—conditions for which existing clinical interventions offer only limited efficacy. The utility
Qiang Shi, Renliang Zhao, Jia Wang, Zhenchen Xiong, Xiangtian Deng, Cheng Zheng, Wenzhi Zhang   +6 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.
Elliott, PM, Lee, P, McKenna, WJ, Sachdev, B, Takenaka, T, Tei, C, Teraguchi, H   +6 more
core   +1 more source

Biomimetic Nanomedicine for Senescence‐Modulated Immune Activation Enhances Immunotherapy Efficacy in Hepatocellular Carcinoma

Advanced Science, EarlyView.
A multifunctional mPDZM nanoplatform is developed in this study. mPDZM integrates chemotherapy‐induced senescence, selective senolysis, and STING‐mediated immune activation. mPDZM effectively clears senescent tumor cells, remodels the tumor immune microenvironment, and enhances antitumor T‐cell responses.
Shiji Fang, Liyun Zheng, Bin Lin, Jiale Chen, Dehai Hou, Yiming Ding, Mengzhu Han, Pan Qin, Mengyuan Wang, Xiaoju Guo, Yeyu Zhang, Gaofeng Shu, Fazong Wu, Jianfei Tu, Minjiang Chen, Zhongwei Zhao, Zhuang Liu, Jiansong Ji   +17 more
wiley   +1 more source

Twenty novel mutations in the alpha-galactosidase A gene causing Fabry disease.

Molecular medicine (Cambridge, Mass.), 2000
Fabry disease, an X-linked inborn error of glycosphingolipid catabolism, results from the deficient activity of the lysosomal exoglycohydrolase alpha-galactosidase A (EC 3.2.1.22; alpha-Gal A). The nature of the molecular lesions in the alpha-Gal A gene in 30 unrelated families was determined to provide precise heterozygote detection, prenatal ...
A K, Topaloglu, G A, Ashley, B, Tong, J, Shabbeer, K H, Astrin, C M, Eng, R J, Desnick   +6 more
openaire   +2 more sources

Senescent Synovial Intimal Fibroblasts Aggravate Osteoarthritis by Regulating Macrophage Polarization and Chondrocyte Phenotype Through ANGPTL4‐α5β1 Axis

Advanced Science, EarlyView.
Senescent synovial intimal fibroblasts (SIF) are identified as key drivers of osteoarthritis. They promote M1 macrophage polarization and cartilage degeneration via the ANGPTL4–α5β1 axis, regulated by transcription factors EGR1 and ATF3. Pharmacological inhibition of this pathway alleviates disease, revealing SIF senescence as a promising therapeutic ...
Muhai Deng, Yunsheng Jiang, Zhiyu Chen, Kaiquan Chen, Na Cao, Yang Huang, Zhengxue Quan, Cheng Chen   +7 more
wiley   +1 more source

Prediction of the responsiveness to pharmacological chaperones: lysosomal human alpha-galactosidase, a case of study

Orphanet Journal of Rare Diseases, 2010
Background The pharmacological chaperones therapy is a promising approach to cure genetic diseases. It relies on substrate competitors used at sub-inhibitory concentration which can be administered orally, reach difficult tissues and have low cost ...
Correra Antonella, Cammisa Marco, Guarracino Mario R, Andreotti Giuseppina, Cubellis Maria   +4 more
doaj   +1 more source

Landscape phage, phage display, stripped phage, biosensors, detection, affinity reagent, nanotechnology, Salmonella typhimurium, Bacillus anthracis

, 2006
Filamentous phage, such as fd used in this study, are thread-shaped bacterial viruses. Their outer coat is a tube formed by thousands equal copies of the major coat protein pVIII. We constructed libraries of random peptides fused to all pVIII domains and
Barbaree, J., Brigati, J., Chen, I-Hsuan, Chin, B., Kouzmitcheva, G., Olsen, Eric V., Petrenko, V., Sorokulova, I., Sykora, J., Vodyanoy, V.   +9 more
core