Fabry disease is an X-linked lysosomal storage disorder caused by GLA mutations, leading to a deficiency in α-Galactosidase A activity and subsequent accumulation of globotriaosylceramide (Gb3). This accumulation contributes to progressive multiorgan dysfunction, with cardiovascular complications, particularly endothelial dysfunction and left ventricular hypertrophy being major drivers of disease morbidity and mortality. Although enzyme replacement therapy is currently the standard treatment, its effectiveness is limited in addressing advanced cardiovascular pathology. To better understand Fabry-associated vascular and cardiac phenotypes, an isogenic human induced pluripotent stem cell (hiPSC) model in which GLA was knocked out was developed using CRISPR/ Cas9. GLA-knockout (GLA-KO) hiPSCs were differentiated into endothelial cells (ECs) and cardiomyocytes (CMs) to evaluate disease-relevant phenotypes in vitro . GLA-KO ECs exhibited normal morphology and differentiation capacity but showed markedly impaired tube formation, high expression of inflammatory genes ICAM1, VCAM1, and SELE, and increased mitochondrial and cytoplasmic reactive oxygen species levels. GLA-KO CMs demonstrated enlarged cell size and nuclear translocation of NFATC4, consistent with hypertrophic remodeling. Together, these findings recapitulate key features of Fabry vasculopathy and cardiomyopathy in a genetically defined, human-derived system. This platform enables direct investigation of Gb3-induced oxidative and inflammatory mechanisms and provides a valuable model for the preclinical evaluation of therapeutic strategies targeting the cardiovascular manifestations of Fabry disease.
‘Seohong’ was bred within the potato breeding program of National Institute of Highland Agriculture (NIHA). It was originated from a cross made in 1997 between ‘Jashim’ as a female and 93K65-1 as a male parent. Seohong was evaluated as a seedling number 97S05-1 for tuber characteristics, growth and yielding ability from 2001 to 2002, and than this clone was renamed as Daegwan 1-82. This clone was evaluated in the regional yield trial and the disease resistance in the five locations for three years. Finally, this clone was named as ‘Seohong’ and registered a new recommended potato cultivar in 2006. This cultivar has a round, shallow eye depth, light red-skinned tuber characteristics and low to medium dry matter content. It showed high resistance to common scab (Streptomyces scabies) and potato leaf roll virus (PLRV), however it was susceptible to potato virus Y (PVY) and late blight (Phytophthora infestans). Its mean tuber yields from the regional yield trial and winter cropping trial were 12 and 49 percent higher than that of ‘Superior’, respectively.
“Haryeong” was bred through the potato breeding program at the National Institute of Highland Agriculture (NIHA). Itin1996. It has a round, smooth, light yelow-skined tuber characteristics and high dry mater content. It showed high resistance tolate bligh