Down-regulation of the mitochondrial i-AAA protease Yme1L induces muscle atrophy via FoxO3a and myostatin activation. [PDF]
J Cell Mol Med, 2020 Muscle atrophy is closely associated with many diseases, including diabetes and cardiac failure. Growing evidence has shown that mitochondrial dysfunction is related to muscle atrophy; however, the underlying mechanisms are still unclear.
Lee YJ, Kim GH, Park SI, Lim JH.
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Loss of the mitochondrial i-AAA protease YME1L leads to ocular dysfunction and spinal axonopathy. [PDF]
EMBO Mol Med, 2019 Disturbances in the morphology and function of mitochondria cause neurological diseases, which can affect the central and peripheral nervous system.
Sprenger HG +11 more
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SPG7 targets the m-AAA protease complex to process MCU for uniporter assembly, Ca2+ influx, and regulation of mitochondrial permeability transition pore opening. [PDF]
J Biol Chem, 2019 The mitochondrial matrix ATPase associated with diverse cellular activities (m-AAA) protease spastic paraplegia 7 (SPG7) has been recently implicated as either a negative or positive regulatory component of the mitochondrial permeability transition pore (
Hurst S, Baggett A, Csordas G, Sheu SS.
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N domain of the Lon AAA+ protease controls assembly and substrate choice. [PDF]
Protein Sci, 2019 : The protein quality control network (pQC) plays critical roles in maintaining protein and cellular homeostasis, especially during stress. Lon is a major pQC AAA+ protease, conserved from bacteria to human mitochondria.
Brown BL +5 more
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The AAA+ protease ClpXP can easily degrade a 31 and a 52-knotted protein. [PDF]
Sci Rep, 2019 Knots in proteins are hypothesized to make them resistant to enzymatic degradation by ATP-dependent proteases and recent studies have shown that whereas ClpXP can easily degrade a protein with a shallow 31 knot, it cannot degrade 52-knotted proteins if ...
Sivertsson EM, Jackson SE, Itzhaki LS.
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Topologically knotted deubiquitinases exhibit unprecedented mechanostability to withstand the proteolysis by an AAA+ protease. [PDF]
Sci Rep, 2018 More than one thousand knotted protein structures have been identified so far, but the functional roles of these knots remain elusive. It has been postulated that backbone entanglement may provide additional mechanostability.
Sriramoju MK, Chen Y, Lee YC, Hsu SD.
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Conformational plasticity of the ClpAP AAA+ protease couples protein unfolding and proteolysis. [PDF]
Nat Struct Mol Biol, 2020 Lopez KE +8 more
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Regulation of mitochondrial plasticity by the i-AAA protease YME1L [PDF]
Biological Chemistry, 2020 Mitochondria are multifaceted metabolic organelles and adapt dynamically to various developmental transitions and environmental challenges. The metabolic flexibility of mitochondria is provided by alterations in the mitochondrial proteome and is tightly ...
Yohsuke Ohba, T. MacVicar, T. Langer
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Molecular insights into the m-AAA protease-mediated dislocation of transmembrane helices in the mitochondrial inner membrane. [PDF]
J Biol Chem, 2017 Protein complexes involved in respiration, ATP synthesis, and protein import reside in the mitochondrial inner membrane; thus, proper regulation of these proteins is essential for cell viability.
Lee S, Lee H, Yoo S, Kim H.
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Discovery of AAA+ Protease Substrates through Trapping Approaches.
Trends in Biochemical Sciences, 2019 Proteases play essential roles in cellular proteostasis. Mechanisms through which proteases recognize their substrates are often hard to predict and therefore require experimentation.
Jui-Yun Rei Liao, K. V. van Wijk
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