AMSH-LP knock out mice cause cystinuria

Research Project

Project/Area Number	23791797
Research Category	Grant-in-Aid for Young Scientists (B)
Allocation Type	Multi-year Fund
Research Field	Urology
Research Institution	Miyagi Cancer Center Research Institute
Principal Investigator	SAKURADA Yu 地方独立行政法人宮城県立病院機構宮城県立がんセンター(研究所), がん先進治療開発研究部, 特任研究員 (70531325)
Project Period (FY)	2011 – 2012
Project Status	Completed (Fiscal Year 2012)
Budget Amount *help	¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000) Fiscal Year 2012: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000) Fiscal Year 2011: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
Keywords	シスチン尿症 / 脱ユビキチン / AMSH-LP / 尿路結石 / 水腎症 / ノックアウトマウス
Research Abstract	To investigate the role on AMSH-LP in vivo, we generated AMSH-LP conditional knock out mice. Surprisingly, hydronephrosis was occurred in one-year old age when AMSH-LP knock out was induced in whole body. Pathologically, urolithiasis was present in hydronephrotic mice. Bladder stone analysis revealed that the stone was consisted of cystine (95%). In urine analysis, the concentration of cystine, ornithine, lysine, and arginine was increased in AMSH-LP knock out mice. Taken together, AMSH-knock out mice were similar to the cystinuria in human.