Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia. - Wikidata - awgldk - TriplyDB

Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia.

Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia.

http://www.wikidata.org/entity/Q42036779

about

Crystal structure of the ATPase domain of the human AAA+ protein paraplegin/SPG7 Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteases Mitochondrial translation and beyond: processes implicated in combined oxidative phosphorylation deficiencies CLUH regulates mitochondrial biogenesis by binding mRNAs of nuclear-encoded mitochondrial proteins Cross Talk of Proteostasis and Mitostasis in Cellular Homeodynamics, Ageing, and Disease Mitochondrial targets for pharmacological intervention in human disease What we have learned from the next-generation sequencing: Contributions to the genetic diagnoses and understanding of pathomechanisms of neurodegenerative diseases NMR structure and MD simulations of the AAA protease intermembrane space domain indicates peripheral membrane localization within the hexaoligomer An intersubunit signaling network coordinates ATP hydrolysis by m-AAA proteases Mitochondrial protein quality control: the mechanisms guarding mitochondrial health Glutamyl-tRNAGln amidotransferase is essential for mammalian mitochondrial translation in vivo Cyclophilin D modulates mitochondrial F0F1-ATP synthase by interacting with the lateral stalk of the complex StAR enhances transcription of genes encoding the mitochondrial proteases involved in its own degradation Autocatalytic processing of m-AAA protease subunits in mitochondria Regulation of OPA1 processing and mitochondrial fusion by m-AAA protease isoenzymes and OMA1 Mouse brain expression patterns of Spg7, Afg3l1, and Afg3l2 transcripts, encoding for the mitochondrial m-AAA protease.Control of mitochondrial integrity in ageing and disease Multifunctional Mitochondrial AAA Proteases Identification and characterization of high molecular weight complexes formed by matrix AAA proteases and prohibitins in mitochondria of Arabidopsis thaliana Modifiers 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 ataxia Electron 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 fragmentation Mitochondrial ribosome assembly in health and disease Quality 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 protease Loss of CLPP alleviates mitochondrial cardiomyopathy without affecting the mammalian UPRmt.RNA-mediated toxicity in neurodegenerative disease The power of yeast to model diseases of the powerhouse of the cell.Caveolin-1 controls mitochondrial function through regulation of m-AAA mitochondrial protease Protein quality control in mitochondria.

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P2860

Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia.

http://www.wikidata.org/entity/Q42036779

description

2006 nî lūn-bûn

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2006年の論文

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2006年論文

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2006年論文

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2006年論文

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2006年論文

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2006年論文

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2006年论文

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2006年论文

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2006年论文

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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

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Molecular and Cellular Biology

P1476

Variable and tissue-specific s ...... hereditary spastic paraplegia.

@en

P2093

Mirko Koppen

http://www.w3.org/2001/XMLSchema#string

Thomas Langer

http://www.w3.org/2001/XMLSchema#string

P2860

OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28

Molecular and functional analyses of the human and mouse genes encoding AFG3L1, a mitochondrial metalloprotease homologous to the human spastic paraplegia protein

Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia

Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease

Mitochondrial DNA mutations in human disease

Additional modules for versatile and economical PCR-based gene deletion and modification in Saccharomyces cerevisiae

The YTA10-12 complex, an AAA protease with chaperone-like activity in the inner membrane of mitochondria.

The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria.

Prohibitins regulate membrane protein degradation by the m-AAA protease in mitochondria.

A novel two-step mechanism for removal of a mitochondrial signal sequence involves the mAAA complex and the putative rhomboid protease Pcp1.

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758-767

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scholarly article

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10.1128/MCB.01470-06

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2

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27

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Elena I Rugarli

Metodi D Metodiev

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2006-11-13T00:00:00Z

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17101804

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1800790

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