TY - JOUR
T1 - Caenorhabditis elegans as a model system to study intercompartmental proteostasis
T2 - Interrelation of mitochondrial function, longevity, and neurodegenerative diseases
AU - Kirstein-Miles, Janine
AU - Morimoto, Richard I.
PY - 2010/5
Y1 - 2010/5
N2 - The protein quality control system, composed of molecular chaperones and proteases, is of vital importance for the maintenance and function of the proteome and the health of the cell. To achieve this, the cellular proteostasis network integrates the protein folding machinery across all compartments of the eukaryotic cell to enable efficient communication and coordinate a rapid response of folding capacity. Quality control in the mitochondria, however, differs from its cytosolic counterpart due to its prokaryotic origin, and is entirely encoded by the nuclear genome. The control and regulatory cross-talk of mitochondrial function in cellular proteostasis is essential for cellular metabolism, organismal development, and lifespan. Consequently, mitochondrial dysfunction has dramatic effects on the development and progression of a number of neurodegenerative diseases, such as Friedreich's ataxia and Parkinson's disease. Studies using Caenorhabditis elegans as a model system have greatly contributed to our current knowledge of inter-compartmental proteostasis on the cellular and organismal levels.
AB - The protein quality control system, composed of molecular chaperones and proteases, is of vital importance for the maintenance and function of the proteome and the health of the cell. To achieve this, the cellular proteostasis network integrates the protein folding machinery across all compartments of the eukaryotic cell to enable efficient communication and coordinate a rapid response of folding capacity. Quality control in the mitochondria, however, differs from its cytosolic counterpart due to its prokaryotic origin, and is entirely encoded by the nuclear genome. The control and regulatory cross-talk of mitochondrial function in cellular proteostasis is essential for cellular metabolism, organismal development, and lifespan. Consequently, mitochondrial dysfunction has dramatic effects on the development and progression of a number of neurodegenerative diseases, such as Friedreich's ataxia and Parkinson's disease. Studies using Caenorhabditis elegans as a model system have greatly contributed to our current knowledge of inter-compartmental proteostasis on the cellular and organismal levels.
KW - Aging
KW - C. elegans
KW - Neurodegenerative diseases
KW - Proteostasis
KW - mtUPR
UR - http://www.scopus.com/inward/record.url?scp=77951554481&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77951554481&partnerID=8YFLogxK
U2 - 10.1002/dvdy.22292
DO - 10.1002/dvdy.22292
M3 - Review article
C2 - 20419784
AN - SCOPUS:77951554481
SN - 1058-8388
VL - 239
SP - 1529
EP - 1538
JO - Developmental Dynamics
JF - Developmental Dynamics
IS - 5
ER -