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Three-dimensional reconstruction of the S885A mutant of human mitochondrial Lon protease
S. Kereïche, L. Kováčik, V. Pevala, L. Ambro, J. Bellová, E. Kutejová, I. Raška
Language English Country Czech Republic
Document type Journal Article, Research Support, Non-U.S. Gov't
NLK
Free Medical Journals
from 2000
Freely Accessible Science Journals
from 2000
ProQuest Central
from 2005-01-01
Health & Medicine (ProQuest)
from 2005-01-01
ROAD: Directory of Open Access Scholarly Resources
from 2000
- MeSH
- Humans MeSH
- Mitochondria enzymology MeSH
- Models, Molecular * MeSH
- Mutant Proteins chemistry MeSH
- Negative Staining MeSH
- Protease La chemistry ultrastructure MeSH
- Imaging, Three-Dimensional * MeSH
- Check Tag
- Humans MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
The Lon protein is a protease belonging to the superfamily of ATPases associated with diverse cellular activities (AAA+). Its main function is the control of protein quality and the maintenance of proteostasis by degradation of misfolded and damaged proteins, which occur in response to numerous stress conditions. It also participates in the regulation of levels of transcription factors that control pathogenesis, development and stress response. We focus our interest on the structure of human mitochondrial Lon (hLon) protease, whose altered expression levels are linked to some severe diseases such as epilepsy, myopathy, or lateral sclerosis. We present the first 3D structure of the ADP-bound human Lon S885A mutant obtained by electron microscopy as a result of preliminary negative staining studies. S885A appears as a hexameric ring of 120 Å diameter having 90 Å in height. Its resolution was estimated at 19 Å by the FSC = 0.5 criterion. This model is a primary step towards the understanding of the mechanism of action of the Lon protease and its involvement in the pathogenesis development.
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- $a Three-dimensional reconstruction of the S885A mutant of human mitochondrial Lon protease / $c S. Kereïche, L. Kováčik, V. Pevala, L. Ambro, J. Bellová, E. Kutejová, I. Raška
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- $a The Lon protein is a protease belonging to the superfamily of ATPases associated with diverse cellular activities (AAA+). Its main function is the control of protein quality and the maintenance of proteostasis by degradation of misfolded and damaged proteins, which occur in response to numerous stress conditions. It also participates in the regulation of levels of transcription factors that control pathogenesis, development and stress response. We focus our interest on the structure of human mitochondrial Lon (hLon) protease, whose altered expression levels are linked to some severe diseases such as epilepsy, myopathy, or lateral sclerosis. We present the first 3D structure of the ADP-bound human Lon S885A mutant obtained by electron microscopy as a result of preliminary negative staining studies. S885A appears as a hexameric ring of 120 Å diameter having 90 Å in height. Its resolution was estimated at 19 Å by the FSC = 0.5 criterion. This model is a primary step towards the understanding of the mechanism of action of the Lon protease and its involvement in the pathogenesis development.
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