Cystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells

scientific article published on April 2006

Cystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1111/J.1460-9568.2006.04686.X
P698PubMed publication ID16623826

P2093author name stringMathieu Lesort
Zhengkuan Mao
Yeun Su Choo
P2860cites workA novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.Q27860836
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutationQ28246858
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal deathQ28287762
Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitroQ28584697
Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubulesQ28590634
Tissue transglutaminase contributes to disease progression in the R6/2 Huntington's disease mouse model via aggregate-independent mechanismsQ28592026
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brainQ29617982
Huntington's disease: a synaptopathy?Q33193898
On the active site thiol of gamma-glutamylcysteine synthetase: relationships to catalysis, inhibition, and regulationQ33566233
Tissue transglutaminase: a possible role in neurodegenerative diseasesQ33881855
Identification of combinatorial drug regimens for treatment of Huntington's disease using Drosophila.Q33928419
Transcriptional dysregulation in Huntington's diseaseQ34001953
Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primatesQ34103541
A quick and simple method for the quantitation of lactate dehydrogenase release in measurements of cellular cytotoxicity and tumor necrosis factor (TNF) activityQ34169361
Therapeutic opportunities in polyglutamine diseaseQ34205521
The role of mitochondrial dysfunction and neuronal nitric oxide in animal models of neurodegenerative diseasesQ34440280
Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's diseaseQ34763178
3-Nitropropionate, the toxic substance of Indigofera , is a suicide inactivator of succinate dehydrogenaseQ35049569
Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nucleiQ36395820
Intracellular heterogeneity in mitochondrial membrane potentials revealed by a J-aggregate-forming lipophilic cation JC-1.Q37494492
Inefficient degradation of truncated polyglutamine proteins by the proteasomeQ37592772
Formation of γ-glutamyl-ε-lysine bridges between membrane proteins by a Ca2+-regulated enzyme in intact erythrocytesQ39244222
Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivationQ40540661
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c releaseQ40551652
Huntington's disease, energy, and excitotoxicityQ40580788
Striatal cells from mutant huntingtin knock-in mice are selectively vulnerable to mitochondrial complex II inhibitor-induced cell death through a non-apoptotic pathwayQ40590116
Mitochondria, free radicals, and neurodegenerationQ41235574
Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia.Q42438521
N(epsilon)-(gamma-L-glutamyl)-L-lysine (GGEL) is increased in cerebrospinal fluid of patients with Huntington's diseaseQ43820046
Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamineQ43873037
'Tissue' transglutaminase ablation reduces neuronal death and prolongs survival in a mouse model of Huntington's diseaseQ44104440
Cystamine inhibits caspase activity. Implications for the treatment of polyglutamine disordersQ44233701
Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in miceQ44316963
Minocycline is protective in a mouse model of Huntington's diseaseQ44697618
Cell-permeable peptide antioxidants targeted to inner mitochondrial membrane inhibit mitochondrial swelling, oxidative cell death, and reperfusion injuryQ44922430
Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregationQ45077697
Mitochondrial defect in Huntington's disease caudate nucleusQ45291734
Transglutaminase activity is related to CAG repeat length in patients with Huntington's diseaseQ45293405
Neuronal loss in layers V and VI of cerebral cortex in Huntington's diseaseQ45293583
Treatment of YAC128 mice and their wild-type littermates with cystamine does not lead to its accumulation in plasma or brain: implications for the treatment of Huntington diseaseQ45297332
Riluzole therapy in Huntington's disease (HD).Q45297539
Cystamine treatment is neuroprotective in the YAC128 mouse model of Huntington disease.Q45298533
Correction of hemophilia B in canine and murine models using recombinant adeno-associated viral vectorsQ45299117
Effect of exogenous and endogenous antioxidants on 3-nitropionic acid-induced in vivo oxidative stress and striatal lesions: insights into Huntington's diseaseQ45301207
Impaired mitochondrial function results in increased tissue transglutaminase activity in situQ45301397
Mechanism for the inhibition of transglutaminase 2 by cystamineQ45302748
Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse modelQ45304699
Epidermal immunization by a needle-free powder delivery technology: Immunogenicity of influenza vaccine and protection in miceQ56772848
P433issue7
P407language of work or nameEnglishQ1860
P921main subjectHuntington's diseaseQ190564
striatumQ1319792
P304page(s)1701-1710
P577publication date2006-04-01
P1433published inEuropean Journal of NeuroscienceQ5412733
P1476titleCystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells
P478volume23

Reverse relations

cites work (P2860)
Q37347572Aberrant Rab11-dependent trafficking of the neuronal glutamate transporter EAAC1 causes oxidative stress and cell death in Huntington's disease
Q42408869Acrolein scavengers, cysteamine and N-benzylhydroxylamine, reduces the mouse liver damage after acetaminophen overdose
Q37078047Brain-derived neurotropic factor/TrkB signaling in the pathogenesis and novel pharmacotherapy of schizophrenia
Q92267922Cysteamine Protects Neurons from Mutant Huntingtin Toxicity
Q39785057Disease-toxicant screen reveals a neuroprotective interaction between Huntington's disease and manganese exposure
Q26822726Dysregulation of glutathione homeostasis in neurodegenerative diseases
Q89497605Exposure to 3-Nitropropionic Acid Mitochondrial Toxin Induces Tau Pathology in Tangle-Mouse Model and in Wild Type-Mice
Q51733341Impact of atypical mitochondrial cyclic-AMP level in nephropathic cystinosis.
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Q39031769Investigational agents for the management of Huntington's disease
Q39673821Matrix Metalloproteinases Are Modifiers of Huntingtin Proteolysis and Toxicity in Huntington's Disease
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Q92537416Therapeutic Applications of Cysteamine and Cystamine in Neurodegenerative and Neuropsychiatric Diseases
Q34487604Therapeutic advances in Huntington's Disease
Q28469280Unbiased gene expression analysis implicates the huntingtin polyglutamine tract in extra-mitochondrial energy metabolism

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