Abstract:
:Homozygous deletion or mutation in the survival motor neuron (SMN)1 gene causes proximal spinal muscular atrophy (SMA), whereas SMN2 acts as a modifying gene that can influence the severity of SMA. It has been suggested that restoration of the SMN protein level in neuronal cells may prevent cell loss and may be helpful for treatment of SMA. Recent studies indicate that the ubiquitin/proteasome pathway is a major system for proteolysis of intracellular proteins. In this study, we investigate whether SMN protein is degraded via the ubiquitin/proteasome pathway. Primary fibroblasts were established from the skin biopsies of SMA patients and the effect of a proteasome inhibitor MG132 and lysosome inhibitor NH(4)Cl on SMN protein level was examined. We found that MG132, but not NH(4)Cl, significantly increased the amount and nuclear accumulation of SMN protein in SMA patient's fibroblasts. Immunoprecipitation/western blot analysis indicated that SMN protein was ubiquitinated in cells. In vitro protein ubiquitination assay also demonstrated that SMN protein could be conjugated with ubiquitin. Taken together, we have provided clear evidences that degradation of SMN protein is mediated via the ubiquitin/proteasome pathway and suggest that proteasome inhibitors may up-regulate SMN protein level and may be useful for the treatment of SMA.
journal_name
Neurochem Intjournal_title
Neurochemistry internationalauthors
Chang HC,Hung WC,Chuang YJ,Jong YJdoi
10.1016/j.neuint.2004.04.005subject
Has Abstractpub_date
2004-12-01 00:00:00pages
1107-12issue
7eissn
0197-0186issn
1872-9754pii
S0197018604000907journal_volume
45pub_type
杂志文章abstract::The possible influence of cholinergic and dopaminergic mechanisms on neurotensin-containing neurones was examined at two different levels; nucleus accumbens and striatum in the rat brain. The acute treatment with the anticholinergic drugs atropine and scopolamine increased neurotensin concentrations in the striatum an...
journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/0197-0186(88)90145-3
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journal_title:Neurochemistry international
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journal_title:Neurochemistry international
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journal_title:Neurochemistry international
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doi:10.1016/j.neuint.2015.06.010
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journal_title:Neurochemistry international
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/0197-0186(90)90045-u
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journal_title:Neurochemistry international
pub_type: 杂志文章
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/j.neuint.2005.02.005
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abstract::Alzheimer disease (AD) is the most common form of adult dementia. Its pathological hallmarks are synaptic degeneration, deposition of amyloid plaques and neurofibrillary tangles, leading to neuronal loss. A few hypotheses have been proposed to explain AD pathogenesis. The beta-amyloid (Abeta) and hyperphosphorylated t...
journal_title:Neurochemistry international
pub_type: 杂志文章,评审
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journal_title:Neurochemistry international
pub_type: 杂志文章
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/j.neuint.2012.11.007
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/0197-0186(81)90008-5
更新日期:1981-01-01 00:00:00
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journal_title:Neurochemistry international
pub_type: 杂志文章
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journal_title:Neurochemistry international
pub_type: 杂志文章
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/j.neuint.2006.08.010
更新日期:2007-01-01 00:00:00
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/j.neuint.2019.02.007
更新日期:2019-05-01 00:00:00
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/s0197-0186(97)00037-5
更新日期:1997-12-01 00:00:00
abstract::TAR DNA-binding protein 43 (TDP-43) is a major pathological protein of ubiquitinated inclusions in motor neurons of sporadic amyotrophic lateral sclerosis (ALS). TDP-43 is ubiquitously expressed and the majority of TDP-43 is normally localized to the nucleus. In motor neurons of patients with ALS, TDP-43 is not locali...
journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/j.neuint.2018.12.009
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/0197-0186(90)90152-j
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/j.neuint.2019.03.013
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journal_title:Neurochemistry international
pub_type: 杂志文章,评审
doi:10.1016/j.neuint.2015.02.001
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/0197-0186(88)90007-1
更新日期:1988-01-01 00:00:00
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/j.neuint.2015.04.009
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/j.neuint.2009.12.004
更新日期:2010-02-01 00:00:00
abstract::The labelling of gangliosides and sialoglycoproteins (SGP) after injecting intraocularly 30 ?Ci of [(3)H]ManNAc was studied in chickens that had previously received, also intraocularly, 30 or 70 nmol of monensin in 10 ?l of ethanol or 24 ?g of colchicine in 10 ?l saline. Controls received ethanol or saline only. Colch...
journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/0197-0186(86)90183-x
更新日期:1986-01-01 00:00:00
abstract::It was previously reported that the K+-evoked release of somatostatin-like immunoreactivity (SRIF-LI) and of cholecystokinin-like immunoreactivity (CCK-LI) from superfused rat cerebrocortical synaptosomes can be enhanced by NMDA or D-serine alone. We here studied the effects of extraterminal pH changes on SRIF-LI and ...
journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/j.neuint.2004.03.005
更新日期:2004-10-01 00:00:00
abstract::It is well established that histaminergic neurons in the posterior hypothalamus make connections with whole brain areas and regulate several functions. Recent evidence indicates that histaminergic neurons are heterogeneous cell group and organized into distinct circuits. However, functional circuits of histaminergic n...
journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/j.neuint.2012.05.022
更新日期:2012-11-01 00:00:00
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journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/j.neuint.2008.11.005
更新日期:2009-02-01 00:00:00
abstract::This review summarizes our present view on the molecular pathogenesis of human (h) E3-deficiency caused by a variety of genetic alterations with a special emphasis on the moonlighting biochemical phenomena related to the affected (dihydro)lipoamide dehydrogenase (LADH, E3, gene: dld), in particular the generation of r...
journal_title:Neurochemistry international
pub_type: 杂志文章,评审
doi:10.1016/j.neuint.2017.05.018
更新日期:2018-07-01 00:00:00
abstract::Alpha-synuclein (ASN), a 140-amino acid protein, is richly expressed in presynaptic terminals in the central nervous system, where it plays a role in synaptic vesicle function. However, if it is altered and accumulated it is involved in neurodegeneration as Parkinson's disease (PD). ASN contained 35-amino acid domain ...
journal_title:Neurochemistry international
pub_type: 杂志文章
doi:10.1016/j.neuint.2006.01.025
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