Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia.
about
Crystal structure of the ATPase domain of the human AAA+ protein paraplegin/SPG7Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteasesMitochondrial translation and beyond: processes implicated in combined oxidative phosphorylation deficienciesCLUH regulates mitochondrial biogenesis by binding mRNAs of nuclear-encoded mitochondrial proteinsCross Talk of Proteostasis and Mitostasis in Cellular Homeodynamics, Ageing, and DiseaseMitochondrial targets for pharmacological intervention in human diseaseWhat we have learned from the next-generation sequencing: Contributions to the genetic diagnoses and understanding of pathomechanisms of neurodegenerative diseasesNMR structure and MD simulations of the AAA protease intermembrane space domain indicates peripheral membrane localization within the hexaoligomerAn intersubunit signaling network coordinates ATP hydrolysis by m-AAA proteasesMitochondrial protein quality control: the mechanisms guarding mitochondrial healthGlutamyl-tRNAGln amidotransferase is essential for mammalian mitochondrial translation in vivoCyclophilin D modulates mitochondrial F0F1-ATP synthase by interacting with the lateral stalk of the complexStAR enhances transcription of genes encoding the mitochondrial proteases involved in its own degradationAutocatalytic processing of m-AAA protease subunits in mitochondriaRegulation of OPA1 processing and mitochondrial fusion by m-AAA protease isoenzymes and OMA1Mouse brain expression patterns of Spg7, Afg3l1, and Afg3l2 transcripts, encoding for the mitochondrial m-AAA protease.Control of mitochondrial integrity in ageing and diseaseMultifunctional Mitochondrial AAA ProteasesIdentification and characterization of high molecular weight complexes formed by matrix AAA proteases and prohibitins in mitochondria of Arabidopsis thalianaModifiers of mutant huntingtin aggregation: functional conservation of C. elegans-modifiers of polyglutamine aggregation.Alternative splicing of Spg7, a gene involved in hereditary spastic paraplegia, encodes a variant of paraplegin targeted to the endoplasmic reticulum.Loss of the m-AAA protease subunit AFG₃L₂ causes mitochondrial transport defects and tau hyperphosphorylation.SPG7 mutations are a common cause of undiagnosed ataxiaElectron cryomicroscopy structure of a membrane-anchored mitochondrial AAA protease.Protein degradation within mitochondria: versatile activities of AAA proteases and other peptidases.OPA1 processing reconstituted in yeast depends on the subunit composition of the m-AAA protease in mitochondria.SPG7 Is an Essential and Conserved Component of the Mitochondrial Permeability Transition Pore.Respiratory dysfunction by AFG3L2 deficiency causes decreased mitochondrial calcium uptake via organellar network fragmentationMitochondrial ribosome assembly in health and diseaseQuality control of mitochondrial protein synthesis is required for membrane integrity and cell fitness.The Mitochondrial m-AAA Protease Prevents Demyelination and Hair Greying.Spastic paraplegia gene 7 in patients with spasticity and/or optic neuropathy.AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival.Quality control of mitochondria: protection against neurodegeneration and ageing.Perrault syndrome is caused by recessive mutations in CLPP, encoding a mitochondrial ATP-dependent chambered proteaseLoss of CLPP alleviates mitochondrial cardiomyopathy without affecting the mammalian UPRmt.RNA-mediated toxicity in neurodegenerative diseaseThe power of yeast to model diseases of the powerhouse of the cell.Caveolin-1 controls mitochondrial function through regulation of m-AAA mitochondrial proteaseProtein quality control in mitochondria.
P2860
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P2860
Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia.
description
2006 nî lūn-bûn
@nan
2006年の論文
@ja
2006年論文
@yue
2006年論文
@zh-hant
2006年論文
@zh-hk
2006年論文
@zh-mo
2006年論文
@zh-tw
2006年论文
@wuu
2006年论文
@zh
2006年论文
@zh-cn
name
Variable and tissue-specific s ...... hereditary spastic paraplegia.
@en
Variable and tissue-specific s ...... hereditary spastic paraplegia.
@nl
type
label
Variable and tissue-specific s ...... hereditary spastic paraplegia.
@en
Variable and tissue-specific s ...... hereditary spastic paraplegia.
@nl
prefLabel
Variable and tissue-specific s ...... hereditary spastic paraplegia.
@en
Variable and tissue-specific s ...... hereditary spastic paraplegia.
@nl
P2860
P50
P356
P1476
Variable and tissue-specific s ...... hereditary spastic paraplegia.
@en
P2093
Mirko Koppen
Thomas Langer
P2860
P304
P356
10.1128/MCB.01470-06
P407
P577
2006-11-13T00:00:00Z