AMSH-LP knock out mice cause cystinuria

AMSH-LP 敲除小鼠导致胱氨酸尿症

• 批准号: 23791797
• 负责人: SAKURADA Yu
• 金额: $ 2.66万
• 依托单位: Miyagi Cancer Center Research Institute
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Young Scientists (B)
• 财政年份: 2011
• 资助国家: 日本
• 起止时间: 2011 至 2012
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-23791797/
• 关键词: 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

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.

为了研究AMSH-LP在体内的作用，我们建立了AMSH-LP条件敲除小鼠。令人惊讶的是，1岁时全身诱导AMSH-LP敲除，出现肾积水。肾积水小鼠病理表现为尿石症。膀胱结石分析显示结石由胱氨酸组成（95%）。在尿液分析中，AMSH-LP敲除小鼠的胱氨酸、鸟氨酸、赖氨酸和精氨酸浓度升高。综上所述，amsh敲除小鼠与人类胱氨酸尿症相似。

项目成果

筋層非浸潤性膀胱癌における経尿道的膀胱腫瘍切除術(TURBT)後塩酸ピラルビシン(THP)3 日間連続膀胱内注入療法の膀胱内再発に関する検討

盐酸吡柔比星（THP）连续膀胱灌注3天治疗非肌层浸润性膀胱癌经尿道膀胱肿瘤切除术（TURBT）后膀胱内复发的研究

• 期刊: 日本泌尿器科学会雑誌(0021-5287)
• 作者: 栃木 達夫;櫻田 祐;青木 大志;川村 貞文;伊藤 しげみ;佐藤 郁郎;立野 紘雄
• 通讯作者: 立野 紘雄