IndraLab

Statements

L-glutamine activates ATXN3. 32 / 32
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"Spinocerebellar ataxia type 3 (SCA3) is caused by an expanded polyglutamine stretch in ataxin-3."
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"For example, SCA3 is caused by abnormal expansion of the polyglutamine in the C-term region of the protein, and the cleavage of the C-term fragment by caspases has been suggested to be essential for pathology of the disease XREF_BIBR."
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"Machado-Joseph disease (MJD, also known as spinocerebellar ataxia type 3, SCA3), an autosomal dominant neurological disorder, is caused by an abnormal expanded polyglutamine (polyQ) repeat in the ataxin-3 protein."
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"Hsp104 Suppresses Polyglutamine Induced Degeneration Post Onset in a Drosophila MJD and SCA3 Model."
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"The deubiquitinating enzyme Ataxin-3 (ATXN3) contains a polyglutamine (PolyQ) region, the expansion of which causes spinocerebellar ataxia type-3 (SCA3)."
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"Spinocerebellar ataxia type 3 (SCA3), the most common spinocerebellar ataxia, is caused by a polyglutamine (polyQ) expansion in the protein ataxin-3 (ATXN3)."
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"A polyglutamine expansion mutation in ataxin-3 causes spinocerebellar ataxia type 3 (SCA3), thereby providing a further link between ubiquitin dependent protein quality control mechanisms and neurodegeneration XREF_BIBR, XREF_BIBR."
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"Spinocerebellar ataxia (also called Machado-Joseph disease) is a late-onset neurodegenerative disease caused by the expansion of polyglutamine (CAG) repeats in the MJD1 gene."
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"SCA1, SCA2, MJD and SCA3, SCA6, SCA, and SCA17 are caused by polyglutamine encoding CAG repeat expansions."
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"Spinocerebellar ataxia type 3 (SCA3, also known as Machado-Joseph disease), a hereditary neurodegenerative disease, is caused by an abnormal expansion of the polyglutamine tract in the causative ATXN3 protein."
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"Treatment with this compound enhanced degradation of proteins important in neurodegeneration such as tau, TDP43 or ataxin-3 (a polyglutamine expanded protein causing SCA3), and could provide novel strategies for increasing protein degradation by regulating protein degradation by the UPS."
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"SCA3 is caused by an expanded polyglutamine tract in the ataxin-3 protein, resulting in conformational changes that lead to toxic gain of function."
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"SCA2 and SCA3 are caused by polyglutamine expansions in ataxin2 and ataxin3, respectively [XREF_BIBR, XREF_BIBR]."
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"Spinocerebellar ataxia 3 (SCA3), a neurodegenerative disease characterized mainly by progressive ataxia affecting balance, gait and speech, is caused by a mutation in the C-terminus polyglutamine (PolyQ) region of the ataxin-3 protein, an ubiquitously expressed deubiquitinating enzyme with important functions in the proteasomal protein degradation pathway and regulation of transcription."
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"SCA3 is caused by an expanded polyglutamine tract in the ataxin-3 protein, resulting in conformational changes that lead to toxic gain of function."
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"Machado-Joseph disease (MJD), the most common dominantly inherited ataxia worldwide, is caused by a polyglutamine (polyQ) expansion in the deubiquitinating (DUB) enzyme ataxin-3."
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"Hsp104 suppresses polyglutamine induced degeneration post onset in a drosophila MJD and SCA3 model."
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"Six of the most frequent dominantly inherited spinocerebellar ataxias (SCAs) worldwide - SCA1, SCA2, SCA3, SCA6, SCA7, and SCA17 - are caused by an expansion of a polyglutamine (polyQ) tract in the corresponding proteins."
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"Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), is autosomal dominant neurodegenerative disease caused by an expansion of polyglutamine encoding CAG repeats in the ATXN3 gene."
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"Spinocerebellar ataxia type 3 (SCA3) is caused by a CAG and polyglutamine repeat expansion in the SCA3 gene."
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"Additionally, it is well acknowledged that the pathogenesis of SCA3 and MJD is involved with pathogenic ataxin-3 induced by expanded polyglutamine repeat in ATXN3."
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"Machado-Joseph disease (MJD) or Spinocerebellar Ataxia type 3 is caused by a polyglutamine encoding CAG expansion in the ATXN3 gene, which encodes a 42 kDa deubiquitinating enzyme (DUB), ataxin-3."
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"More studies are required to develop therapeutic agents for treating spinocerebellar ataxia type 3 (SCA3), which is caused by mutant polyglutamine expanded ataxin-3 and is the most prevalent subtype of spinocerebellar ataxias."
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"Decreased ataxin-3 cleavage may result in decreased formation of neurotoxic ataxin-3 protein aggregates, through decreased presence of the lone aggregation prone polyglutamine containing peptide."
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"Finally, we link this novel function of ataxin-3 to molecular events that may contribute to SCA3 disease pathogenesis : polyglutamine expansion increases the affinity of ataxin-3 for CHIP, which correlates with decreased levels of this neuroprotective E3 in a mouse model of SCA3."
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"A peptide inhibitor of polyglutamine aggregation, known as polyQ binding peptide 1, has been shown previously to prevent the maturation of ataxin-3 fibrils."
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"SCA3 is caused by polyglutamine expansion in ataxin-3."
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"Spinocerebellar ataxia type 3 (SCA3), or Machado-Joseph disease (MJD), is caused by the expansion of a polyglutamine repeat in the ataxin-3 protein."
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"The ATXN3 targeting ASO achieved sustained reduction of polyglutamine expanded ATXN3 up to 8 weeks after treatment and prevented oligomeric and nuclear accumulation of ATXN3 up to at least 14 weeks after treatment."
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"For example, spinocerebellar ataxia type 3 (SCA3), which is characterized by progressive dysfunction of the cerebellum and brain stem, is caused by polyglutamine (polyQ) expansion in the ataxin-3 gene."
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"SCA3 is caused by a polyglutamine encoding CAG repeat expansion in the ATXN3 gene."
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"SCA3 is caused by polyglutamine expansion of the Ataxin-3 gene and is the most common inherited cerebellar ataxia in some populations [XREF_BIBR]."
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