Multifunctional Mitochondrial AAA Proteases
نویسندگان
چکیده
منابع مشابه
Multifunctional Mitochondrial AAA Proteases
Mitochondria perform numerous functions necessary for the survival of eukaryotic cells. These activities are coordinated by a diverse complement of proteins encoded in both the nuclear and mitochondrial genomes that must be properly organized and maintained. Misregulation of mitochondrial proteostasis impairs organellar function and can result in the development of severe human diseases. ATP-dr...
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An ubiquitous and conserved proteolytic system regulates the stability of mitochondrial inner membrane proteins. Two AAA proteases with catalytic sites at opposite membrane surfaces form a membrane-integrated quality control system and exert crucial functions during the biogenesis of mitochondria. Their activity is modulated by another membrane-protein complex that is composed of prohibitins. P...
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The existence of a proteolytic system which can specifically recognize and cleave proteins in mitochondria is now well established. Components of this system comprise processing peptidases, ATP-dependent peptidases, and oligopeptidases. Processing peptidases mediate limited proteolysis while ATP-dependent proteases and oligopeptidases, working sequentially, are able to degrade proteins to amino...
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We studied presequence processing of the mitochondrial-matrix targeted acetohydroxyacid synthase (Ilv2). C-terminal 3HA-tagging altered the cleavage pattern from a single step to sequential two-step cleavage, giving rise to two Ilv2-3HA forms (A and B). Both cleavage events were dependent on the mitochondrial processing peptidase (MPP). We present evidence for the involvement of three AAA ATPas...
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The human YME1L protease is a membrane-anchored AAA+ enzyme that controls proteostasis at the inner membrane and intermembrane space of mitochondria. Understanding how YME1L recognizes substrates and catalyses ATP-dependent degradation has been hampered by the presence of an insoluble transmembrane anchor that drives hexamerization of the catalytic domains to form the ATPase active sites. Here,...
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ژورنال
عنوان ژورنال: Frontiers in Molecular Biosciences
سال: 2017
ISSN: 2296-889X
DOI: 10.3389/fmolb.2017.00034