TY - JOUR
T1 - Matrix proteases in mitochondrial DNA function
AU - Matsushima, Yuichi
AU - Kaguni, Laurie S.
N1 - Funding Information:
This work was supported by National Institutes of Health Grant GM45295 (to L.S.K.), and a Grant-in-Aid for Scientific Research (C) from Japan Society for the Promotion of Science (to Y.M.).
PY - 2012/9
Y1 - 2012/9
N2 - Lon, ClpXP and m-AAA are the three major ATP-dependent proteases in the mitochondrial matrix. All three are involved in general quality control by degrading damaged or abnormal proteins. In addition to this role, they are proposed to serve roles in mitochondrial DNA functions including packaging and stability, replication, transcription and translation. In particular, Lon has been implicated in mtDNA metabolism in yeast, fly and humans. Here, we review the role of Lon protease in mitochondrial DNA functions, and discuss a putative physiological role for mitochondrial transcription factor A (TFAM) degradation by Lon protease. We also discuss the possible roles of m-AAA and ClpXP in mitochondrial DNA functions, and the putative candidate substrates for the three matrix proteases. This article is part of a Special Issue entitled: Mitochondrial Gene Expression.
AB - Lon, ClpXP and m-AAA are the three major ATP-dependent proteases in the mitochondrial matrix. All three are involved in general quality control by degrading damaged or abnormal proteins. In addition to this role, they are proposed to serve roles in mitochondrial DNA functions including packaging and stability, replication, transcription and translation. In particular, Lon has been implicated in mtDNA metabolism in yeast, fly and humans. Here, we review the role of Lon protease in mitochondrial DNA functions, and discuss a putative physiological role for mitochondrial transcription factor A (TFAM) degradation by Lon protease. We also discuss the possible roles of m-AAA and ClpXP in mitochondrial DNA functions, and the putative candidate substrates for the three matrix proteases. This article is part of a Special Issue entitled: Mitochondrial Gene Expression.
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U2 - 10.1016/j.bbagrm.2011.11.008
DO - 10.1016/j.bbagrm.2011.11.008
M3 - Review article
C2 - 22172992
AN - SCOPUS:84864310340
SN - 1874-9399
VL - 1819
SP - 1080
EP - 1087
JO - Biochimica et Biophysica Acta - Gene Regulatory Mechanisms
JF - Biochimica et Biophysica Acta - Gene Regulatory Mechanisms
IS - 9-10
ER -