Bladder outlet obstruction in male cystinuria mice.
Bladder outlet obstruction in male cystinuria mice.
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DOI：
10.1007/s11255-009-9597-y
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发表时间：
2010-03
影响因子：
2
通讯作者：
Levin, Robert M.
中科院分区：
文献类型：
--
作者：
Ercolani, Mathew;Sahota, Amrik;Schuler, Catherine;Yang, Min;Evan, Andrew P.;Reimer, David;Barone, Joseph G.;Tischfield, Jay A.;Levin, Robert M.
胱氨酸尿症是儿童尿路结石最常见的遗传原因。它可导致梗阻性尿病，这是肾功能衰竭的主要原因。遗传学研究已确定SLC3A1和SLC7A9两个基因直接参与胱氨酸结石的形成。Slc3a1基因敲除的雄性小鼠在膀胱中出现胱氨酸结石，并且在较小程度上在肾脏中出现胱氨酸结石。Slc3a1基因敲除的雌性小鼠也会出现胱氨酸尿症，但它们不会形成结石。本研究的具体目的是表征胱氨酸尿症小鼠的膀胱功能。以4 ~ 5月龄C57B/129混合菌株背景小鼠8只（雄性4只，雌性4只）和16只（雄性9只，雌性7只）Slc3a1敲除小鼠为研究对象。每只小鼠麻醉后，膀胱穹窿置管进行膀胱切开术。膀胱术后，立即切除膀胱，称重，并将其分成三条全厚度的条带进行收缩研究。患有胱氨酸尿症的雄性小鼠膀胱重量显著增加，均有结石，顺应性下降，对电场刺激、ATP、乙醇和KCl的收缩反应减弱。与对照组相比，雌性基因敲除小鼠膀胱重量正常，排尿压力下降，顺应性略有下降，收缩反应略有下降。这些研究清楚地表明，膀胱结石的发展，在雄性膀胱尿鼠导致部分出口阻塞。雌性膀胱尿鼠虽无膀胱结石，但膀胱功能轻度受损；大概是由于胱氨酸晶体的存在。
Cystinuria is the most common inherited cause of urinary tract stones in children. It can lead to obstructive uropathy, which is a major cause of renal failure. Genetic studies have identified two genes, SLC3A1 and SLC7A9, to be directly involved in cystine stone formation. Slc3a1 knockout male mice develop cystine stones in the bladder and, to a lesser extent, in the kidney. Slc3a1 knockout female mice also develop cystinuria, but they do not form stones. The specific aim of this study was to characterize bladder function in cystinuria mice. Eight control (4 male, 4 female) and 16 Slc3a1 knockout (9 male, 7 female) mice of mixed strain background (C57B/129, age 4–5 months) were evaluated. Each mouse was anesthetized and the bladder dome catheterized for cystometry. Immediately following cystometry, the bladder was excised, weighed, and separated into three full thickness strips for contractile studies. Bladders from cystinuria male mice had significantly increased weight, all of them had stones, decreased compliance, and decreased contractile responses to field stimulation, ATP, carbachol, and KCl. Compared with controls, female knockout mice showed normal bladder weight, decreased voiding pressure, slightly decreased compliance, and slightly decreased contractile responses. These studies clearly demonstrate that the bladder stones that developed in the male cystinuria mice resulted in a partial outlet obstruction. Although the female cystinuria mice did not have bladder stones, bladder function was mildly impaired; presumably by the presence of cystine crystals.
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影响因子：
19.6
作者：
Botzenhart, E;Vester, U;Eggermann, T
通讯作者：
Eggermann, T
影响因子：
3
作者：
Dello Strologo, Luca;Laurenzi, Chiara;Pastore, Anna
通讯作者：
Pastore, Anna
影响因子：
1.6
作者：
Schwentner, C;Oswald, J;Radmayr, C
通讯作者：
Radmayr, C
DOI：
10.1152/ajprenal.1998.275.1.f154
发表时间：
1998-07-01
影响因子：
4.2
作者：
Stockelman, MG;Lorenz, JN;Stambrook, PJ
通讯作者：
Stambrook, PJ
影响因子：
3.5
作者：
Feliubadaló, L;Arbonés, ML;Nunes, V
通讯作者：
Nunes, V