The mitochondrial and kidney disease phenotypes of kd/kd mice under germfree conditions

The mitochondrial and kidney disease phenotypes of kd/kd mice under germfree conditions
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DOI:
10.1016/j.jaut.2005.10.006
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发表时间:
2006-02-01
影响因子:
12.8
通讯作者:
Gasser, DL
Gasser, DL
中科院分区:
医学1区
文献类型:
--
作者:
Hallman, TM;Peng, M;Gasser, DL

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间质性肾炎是kd/kd小鼠自发发生的疾病,但其发病机制尚未完全阐明。表型的最早表现是小鼠42天龄时线粒体出现超微结构缺陷。为了考察环境对表型的影响,将纯合子B6.kd/kd小鼠从特定的无病原体(SPF)环境转移到无菌环境(GF),并观察疾病的发展。GF状态导致肾小管间质肾炎的发生率显著降低。此外,GF条件显著减少了线粒体表型的出现,在出生至155天的GF小鼠中没有线粒体异常的迹象。这些结果表明,环境因素参与了这种疾病表型所有已知表现的进展。(C)2005爱思唯尔有限公司。保留所有权利。
Interstitial nephritis occurs spontaneously in kd/kd mice, but the mechanisms leading to this disease have not been fully elucidated. The earliest manifestation of a phenotype is the appearance of ultrastructural defects in the mitochondria of mice as young as 42 days of age. To examine the influence of the environment on the phenotype, homozygous B6.kd/kd mice were transferred from specific pathogen-free (SPF) conditions to a germfree (GF) environment, and the development of the disease was observed. The GF state resulted in a highly significant reduction in the frequency of tubulointerstitial nephritis. In addition, GF conditions markedly reduced the appearance of the mitochondrial phenotype, with no sign of mitochondrial abnormalities in GF mice of up to 155 days of age. These results suggest that environmental factors are involved in the progression of all known manifestations of this disease phenotype. (c) 2005 Elsevier Ltd. All rights reserved.