Decreased expression of the mitochondrial matrix proteases Lon and ClpP in cells from a patient with hereditary spastic paraplegia (SPG13).
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Mutated desmoglein-2 proteins are incorporated into desmosomes and exhibit dominant-negative effects in arrhythmogenic right ventricular cardiomyopathyIs Modulation of Oxidative Stress an Answer? The State of the Art of Redox Therapeutic Actions in Neurodegenerative DiseasesMultitasking in the mitochondrion by the ATP-dependent Lon proteaseMitochondrial protein quality control: the mechanisms guarding mitochondrial healthQuantitative proteomics reveals cellular targets of celastrolControl of mitochondrial integrity in ageing and diseaseHereditary spastic paraplegia: clinico-pathologic features and emerging molecular mechanisms.Alternative oxidase dependent respiration leads to an increased mitochondrial content in two long-lived mutants of the aging model Podospora anserinaBiological roles of the Podospora anserina mitochondrial Lon protease and the importance of its N-domain.Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors.Upregulation of the mitochondrial Lon Protease allows adaptation to acute oxidative stress but dysregulation is associated with chronic stress, disease, and aging.Mitochondrial Lon protease at the crossroads of oxidative stress, ageing and cancer.Inactivation of the hereditary spastic paraplegia-associated Hspd1 gene encoding the Hsp60 chaperone results in early embryonic lethality in mice.Unfolded protein stress in the endoplasmic reticulum and mitochondria: a role in neurodegenerationMitochondrial ClpP activity is required for cisplatin resistance in human cells.Perrault syndrome is caused by recessive mutations in CLPP, encoding a mitochondrial ATP-dependent chambered proteaseEffects of a Mutation in the HSPE1 Gene Encoding the Mitochondrial Co-chaperonin HSP10 and Its Potential Association with a Neurological and Developmental Disorder.Mitochondrial function controls intestinal epithelial stemness and proliferation.The multifaceted role of Lon proteolysis in seedling establishment and maintenance of plant organelle function: living from protein destruction.Mitochondrial proteomics--a tool for the study of metabolic disorders.DNA and RNA analysis of blood and muscle from bodies with variable postmortem intervals.Mitochondrial cereblon functions as a Lon-type protease.Down-regulation of the mitochondrial matrix peptidase ClpP in muscle cells causes mitochondrial dysfunction and decreases cell proliferation.Mitochondrial Quality Control Proteases in Neuronal Welfare.A cell model to study different degrees of Hsp60 deficiency in HEK293 cells.SMN2 exon 7 splicing is inhibited by binding of hnRNP A1 to a common ESS motif that spans the 3' splice site.The presence of enterovirus, adenovirus, and parvovirus B19 in myocardial tissue samples from autopsies: an evaluation of their frequencies in deceased individuals with myocarditis and in non-inflamed control hearts.Cellular consequences of oxidative stress in riboflavin responsive multiple acyl-CoA dehydrogenation deficiency patient fibroblasts.The Hsp60 folding machinery is crucial for manganese superoxide dismutase folding and function.Mitochondrial Chaperones in the Brain: Safeguarding Brain Health and Metabolism?Mapping interactions with the chaperone network reveals factors that protect against tau aggregationPerrault syndrome type 3 caused by diverse molecular defects in CLPP
P2860
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P2860
Decreased expression of the mitochondrial matrix proteases Lon and ClpP in cells from a patient with hereditary spastic paraplegia (SPG13).
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年学术文章
@wuu
2008年学术文章
@zh-cn
2008年学术文章
@zh-hans
2008年学术文章
@zh-my
2008年学术文章
@zh-sg
2008年學術文章
@yue
2008年學術文章
@zh
2008年學術文章
@zh-hant
name
Decreased expression of the mi ...... ry spastic paraplegia (SPG13).
@en
Decreased expression of the mi ...... hereditary spastic paraplegia
@nl
type
label
Decreased expression of the mi ...... ry spastic paraplegia (SPG13).
@en
Decreased expression of the mi ...... hereditary spastic paraplegia
@nl
prefLabel
Decreased expression of the mi ...... ry spastic paraplegia (SPG13).
@en
Decreased expression of the mi ...... hereditary spastic paraplegia
@nl
P2093
P1433
P1476
Decreased expression of the mi ...... ry spastic paraplegia (SPG13).
@en
P2093
B Fontaine
J H Christensen
J Palmfeldt
M N Nielsen
N Gregersen
P304
P356
10.1016/J.NEUROSCIENCE.2008.01.070
P407
P577
2008-02-19T00:00:00Z