Mitochondrial Respiration Is Impaired during Late-Stage Hamster Prion Infection.
Mitochondrial Respiration Is Impaired during Late-Stage Hamster Prion Infection.
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晚期仓鼠朊病毒感染期间线粒体呼吸受损。
DOI：
10.1128/jvi.00524-17
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发表时间：
2017
影响因子：
5.4
通讯作者：
Priola,SuzetteA
中科院分区：
文献类型：
--
作者：
Faris,Robert;Moore,RogerA;Ward,Anne;Sturdevant,DanE;Priola,SuzetteA
Mitochondria are crucial to proper neuronal function and overall brain health. Mitochondrial dysfunction within the brain has been observed in many neurodegenerative diseases, including prion disease. Several markers of decreased mitochondrial activity during prion infection have been reported, yet the bioenergetic respiratory status of mitochondria from prion-infected animals is unknown. Here we show that clinically ill transgenic mice overexpressing hamster prion protein (Tg7) infected with the hamster prion strain 263K suffer from a severe deficit in mitochondrial oxygen consumption in response to the respiratory complex II substrate succinate. Characterization of the mitochondrial proteome of purified brain mitochondria from infected and uninfected Tg7 mice showed significant differences in the relative abundance of key mitochondrial electron transport proteins in 263K-infected animals relative to that in controls. Our results suggest that at clinical stages of prion infection, dysregulation of respiratory chain proteins may lead to impairment of mitochondrial respiration in the brain.IMPORTANCEMitochondrial dysfunction is present in most major neurodegenerative diseases, and some studies have suggested that mitochondrial processes may be altered during prion disease. Here we show that hamster prion-infected transgenic mice overexpressing the hamster prion protein (Tg7 mice) suffer from mitochondrial respiratory deficits. Tg7 mice infected with the 263K hamster prion strain have little or no signs of mitochondrial dysfunction at the disease midpoint but suffer from a severe deficit in mitochondrial respiration at the clinical phase of disease. A proteomic analysis of the isolated brain mitochondria from clinically affected animals showed that several proteins involved in electron transport, mitochondrial dynamics, and mitochondrial protein synthesis were dysregulated. These results suggest that mitochondrial dysfunction, possibly exacerbated by prion protein overexpression, occurs at late stages during 263K prion disease and that this dysfunction may be the result of dysregulation of mitochondrial proteins.
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DOI：
10.1111/j.1749-6632.1980.tb50764.x
发表时间：
1980-01-01
影响因子：
5.2
作者：
Gautherie, M
通讯作者：
Gautherie, M
厘米和毫米波长的热像图成像 *
影响因子：
5.2
作者：
J. Edrich;W. Jobe;R. Cacak;W. Hendee;C. J. Smyth;M. Gautherie;C. Gros;R. Zimmer;J. Robert;P. Thouvenot;J. Escanye;C. Itty
通讯作者：
C. Itty
DOI：
--
发表时间：
1978
期刊：
影响因子：
--
作者：
R. Kingston
通讯作者：
R. Kingston
DOI：
10.1111/j.1749-6632.1980.tb50754.x
发表时间：
1980-01-01
影响因子：
5.2
作者：
Cetas, T C;Connor, W G;Manning, M R
通讯作者：
Manning, M R
用于组织分析的双光束声学系统。
影响因子：
3.5
作者：
T. Sachs;C. D. Janney
通讯作者：
C. D. Janney