Generation of a human induced pluripotent stem cell line, BRCi009-A, derived from a patient with glycogen storage disease type 1a.

Glycogen storage disease type 1a (GSD1a) is an autosomal recessive disorder caused by mutations of the glucose-6-phosphatase (G6PC) gene. Mutations of the G6PC gene lead to excessive accumulation of glycogen in the liver, kidney, and intestinal mucosa due to the deficiency of microsomal glucose-6-phosphatase. Human induced pluripotent stem cells (iPSCs) enable the production of patient-derived hepatocytes in culture and are therefore a promising tool for modeling GSD1a.

Spatial distribution and temporal variation of Microcystis species composition and microcystin concentration in Lake Biwa.

Spatial and temporal variation in Microcystis species composition and microcystin concentration, quantified by enzyme-linked immunosorbent assay and high-performance liquid chromatography, were investigated during a 3-year period (1998-2000) in the Northern Basin of Lake Biwa. The Northern Basin generally had a concentration of 5 microg L(-1) or less, except at station 1 (Nagahama Bay) from July to October during the study period. The maximum concentration at station 1 was 22.