IndraLab

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L-glutamine activates ATXN3. 43 / 43
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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."
32962458
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"Hsp104 Suppresses Polyglutamine Induced Degeneration Post Onset in a Drosophila MJD and SCA3 Model."
24039611
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"Spinocerebellar ataxia type 3 (SCA3) is caused by an expanded polyglutamine stretch in ataxin-3."
34938609
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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 the most common form of spinocerebellar ataxia worldwide, caused by a polyglutamine (polyQ) repeat at the Ataxin-3 (ATX3) C-terminus."
39063148
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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), the most common dominantly inherited ataxia worldwide, is caused by a polyglutamine (polyQ) expansion in the deubiquitinating (DUB) enzyme ataxin-3."
20940148
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"Machado-Joseph disease (MJD) is an autosomal dominant spinocerebellar ataxia (SCA) caused by a polyglutamine expansion in the ataxin-3 protein, which initiates a cascade of pathogenic events, including transcriptional dysregulation."
38900099
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"MJD/SCA3, the most common form of dominant Spinocerebellar Ataxia worldwide, is caused by an expansion of the polyglutamine tract within the ataxin-3 protein, and is characterized by motor impairments."
38404918
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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]."
25108806
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"SCA1, SCA2, MJD and SCA3, SCA6, SCA, and SCA17 are caused by polyglutamine encoding CAG repeat expansions."
21626557
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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."
29908063
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"SCA3/MJD, the most frequent neurodegenerative ataxia worldwide, is caused by the abnormal expansion of the polyglutamine tract (polyQ) at ataxin-3."
39941093
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"SCA3/MJD is caused by an extended polyglutamine coding repeat sequence in the disease gene and is one of the glutamine family of diseases, in 1994, Kawaguchi et al. cloned the pathogenic gene into 14q[MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
38749872
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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."
22970133
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"SCA3 is caused by polyglutamine expansion in ataxin-3."
32955441
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"SCA3 is caused by an expansion of the C-terminal polyglutamine (polyQ) tract in the ATXN3 protein."
38612794
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"Polyglutamine-expanded ataxin-3-Q79 causes the ataxic symptom of SCA3 transgenic mouse."
26254860
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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."
| PMC
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"MJD is caused by a polyglutamine (PolyQ)-encoding CAG repeat expansion in the MJD1 gene (ATXN3), which leads to aggregation of the polyQ fragment, predominantly in neurons."
22868174
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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."
32361312
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"As one of the nine polyglutamine neurodegenerative diseases, SCA3 is caused by an abnormally expanded polyglutamine (polyQ) repeat in the ataxin-3 protein."
35851059
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"SCA3 is caused by expansion of a polyglutamine (polyQ)-encoding CAG trinucleotide repeat in the ATXN3 gene (Kawaguchi et al., 1994), which encodes the ataxin-3 protein (ATXN3)."
37122622
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"Spinocerebellar ataxia type 3 (SCA3), caused by the abnormal expansion of polyglutamine (polyQ) in the ataxin-3 protein, is one of the inherited polyQ neurodegenerative diseases that share similar genetic and molecular features."
39765823
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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."
31231176
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"Spinocerebellar ataxia type 3 (SCA3) is caused by a CAG and polyglutamine repeat expansion in the SCA3 gene."
10369861
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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."
31683630
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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."
21855799
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"In SCA3, a polyglutamine expansion in ATXN3 causes neuron loss in disease-vulnerable brain regions, resulting in progressive loss of coordination and ultimately death."
39900303
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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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"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."
26331033
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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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"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."
22008259
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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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"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."
33483467
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"SCA2 and SCA3 are caused by polyglutamine expansions in ataxin2 and ataxin3, respectively [XREF_BIBR, XREF_BIBR]."
20383523
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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."
19699305
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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."
22749051
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"Huntington’s disease (HD) and spinocerebellar ataxia type 3 (SCA3, also called Machado-Joseph disease) are caused by extensive polyglutamine (polyQ) expansion of the huntingtin or ataxin-3 protein [334]."
37545006
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"MJD is caused by an expansion of a polyglutamine (polyQ)-encoding CAG repeat in the ATXN3 gene [2]."
37408238
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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."
26123252
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"SCA3 is caused by a polyglutamine encoding CAG repeat expansion in the ATXN3 gene."
21704388
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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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