Imbalanced Production of Reactive Oxygen Species and Mitochondrial Antioxidant SOD2 in Fabry Disease-Specific Human Induced Pluripotent Stem Cell-Differentiated Vascular Endothelial Cells

Autor: Wei-Lien Tseng, Shih-Jie Chou, Huai-Chih Chiang, Mong-Lien Wang, Chian-Shiu Chien, Kuan-Hsuan Chen, Hsin-Bang Leu, Chien-Ying Wang, Yuh-Lih Chang, Yung-Yang Liu, Yuh-Jyh Jong, Shinn-Zong Lin, Shih-Hwa Chiou, Shing-Jong Lin, Wen-Chung Yu

Publikováno v: Cell Transplantation, Vol 26 (2017)

Fabry disease (FD) is an X-linked inherited lysosomal storage disease caused by α-galactosidase A (GLA) deficiency. Progressive intracellular accumulation of globotriaosylceramide (Gb3) is considered to be pathogenically responsible for the phenotyp

Externí odkaz: https://doaj.org/article/23c8f644a4d448c19f9704cc3a96b400

Laminin modification subretinal bio-scaffold remodels retinal pigment epithelium-driven microenvironment in vitro and in vivo

Autor: Kuo Hsuan Hung, Wei Lien Tseng, Shih Jen Chen, Mong Lien Wang, De Kuang Hwang, Yong Yu Jhan, Chia Hsien Hsu, Ke Hung Chien, Shih Hwa Chiou, Tai Chi Lin, Yu Chien Chung, Jen Hua Chuang, Chi Hsien Peng, Chao Kuei Lee, Chia-Ching Chang

Publikováno v: Oncotarget

// Chi-Hsien Peng 1,3,8,* , Jen-Hua Chuang 2,9,* , Mong-Lien Wang 2,10,* , Yong-Yu Jhan 1,9 , Ke-Hung Chien 4,9 , Yu-Chien Chung 1,10 , Kuo-Hsuan Hung 1,8,10 , Chia-Ching Chang 5 , Chao-Kuei Lee 6 , Wei-Lien Tseng 2,9 , De-Kuang Hwang 1,10 , Chia-Hsi

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::12952f5587d8f8476d2b9d94171ad733
https://doi.org/10.18632/oncotarget.11502

Poly(ADP-Ribose) Polymerase 1: Cellular Pluripotency, Reprogramming, and Tumorogenesis

Autor: Wei Lien Tseng, Yen-Jen Sung, Shih Hwa Chiou, Hsin Yang Li, Yuh Lih Chang, Bo Hua Jiang, Mong Lien Wang

Publikováno v: International Journal of Molecular Sciences
International Journal of Molecular Sciences, Vol 16, Iss 7, Pp 15531-15545 (2015)

Poly(ADP-ribos)ylation (PARylation) is the catalytic function of the Poly(ADP-ribose) polymerases (Parps) family for post-translational modification in cellular process. Being a major member of Parps, Parp1 is a crucial nuclear factor with biological

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::814f2eae47a6dac9ce7f17f2e10f45b6
http://europepmc.org/articles/PMC4519911

Using CRISPR/Cas9-Mediated GLA Gene Knockout as an In Vitro Drug Screening Model for Fabry Disease

Autor: Huai Chih Chiang, Hsin Bang Leu, Yuh-Jyh Jong, Chian Shiu Chien, Mong Lien Wang, Hui Yung Song, Wei Lien Tseng, Yi Ping Yang, Chung-Hsuan Chen, Wen Chung Yu, Shih Hwa Chiou, Ping Wu

Publikováno v: International Journal of Molecular Sciences; Volume 17; Issue 12; Pages: 2089
International Journal of Molecular Sciences, Vol 17, Iss 12, p 2089 (2016)
International Journal of Molecular Sciences

The CRISPR/Cas9 Genome-editing system has revealed promising potential for generating gene mutation, deletion, and correction in human cells. Application of this powerful tool in Fabry disease (FD), however, still needs to be explored. Enzyme replace

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1688621a364d3538089b0b47996aa95d

Integrin-mediated Membrane Blebbing Is Dependent on Sodium-Proton Exchanger 1 and Sodium-Calcium Exchanger 1 Activity

Autor: Chi Hung Lin, Wei Lien Tseng, Chih Yung Tang, Szecheng J. Lo, Tien Shen Lew, Ching-Ping Tseng, Yung Hsiang Yi, Yu-Sun Chang

Publikováno v: Journal of Biological Chemistry. 287:10316-10324

Integrin signaling and membrane blebbing modulate cell adhesion, spreading, and migration. However, the relationship between integrin signaling and membrane blebbing is unclear. Here, we show that an integrin-ligand interaction induces both membrane

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3ca2d85bdc862c5104a34424d4e01684
https://doi.org/10.1074/jbc.m111.244962