Results 21 to 30 of about 16,136 (210)
Pharmaceutics, 2021 Seven of the most frequent spinocerebellar ataxias (SCAs) are caused by a pathological expansion of a cytosine, adenine and guanine (CAG) trinucleotide repeat located in exonic regions of unrelated genes, which in turn leads to the synthesis of ...
Fabiola V. Borbolla-Jiménez +5 more
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eLife, 2016 The polyglutamine expansion in huntingtin protein causes Huntington’s disease. Here, we investigated structural and biochemical properties of huntingtin and the effect of the polyglutamine expansion using various biophysical experiments including ...
Ravi Vijayvargia +13 more
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Acta Biochimica et Biophysica Sinica, 2023 Nine polyglutamine (polyQ) proteins have already been identified that are considered to be associated with the pathologies of neurodegenerative disorders called polyQ diseases, but whether these polyQ proteins mutually interact and synergize in ...
Hong Jun-Ye +6 more
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Pathologic polyglutamine aggregation begins with a self-poisoning polymer crystal
eLife, 2023 A long-standing goal of amyloid research has been to characterize the structural basis of the rate-determining nucleating event. However, the ephemeral nature of nucleation has made this goal unachievable with existing biochemistry, structural biology ...
Tej Kandola +13 more
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Solution structure of polyglutamine tracts in GST‐polyglutamine fusion proteins
FEBS Letters, 2002 Aggregation of expanded polyglutamine (polyQ) seems to be the cause of various genetic neurodegenerative diseases. Relatively little is known as yet about the polyQ structure and the mechanism that induces aggregation. We have characterised the solution structure of polyQ in a proteic context using a model system based on glutathione S‐transferase ...
Masino L +5 more
openaire +3 more sources
Biology, 2023 Removal of the 5′ cap structure of RNAs (termed decapping) is a pivotal event in the life of cytoplasmic mRNAs mainly catalyzed by a conserved holoenzyme, composed of the catalytic subunit DCP2 and its essential cofactor DCP1.
Fivos Borbolis +6 more
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Polyglutamine tracts regulate autophagy [PDF]
Autophagy, 2017 Expansions of polyglutamine (polyQ) tracts in different proteins cause 9 neurodegenerative conditions, such as Huntington disease and various ataxias. However, many normal mammalian proteins contain shorter polyQ tracts. As these are frequently conserved in multiple species, it is likely that some of these polyQ tracts have important but unknown ...
Rubinsztein, DC +10 more
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The role of ubiquitination in spinal and bulbar muscular atrophy
Frontiers in Molecular Neuroscience, 2022 Spinal and bulbar muscular atrophy (SBMA) is a neurodegenerative and neuromuscular genetic disease caused by the expansion of a polyglutamine-encoding CAG tract in the androgen receptor (AR) gene.
Medha Sengupta +2 more
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d-Polyglutamine Amyloid Recruits l-Polyglutamine Monomers and Kills Cells [PDF]
Journal of Molecular Biology, 2014 Polyglutamine (polyQ) amyloid fibrils are observed in disease tissue and have been implicated as toxic agents responsible for neurodegeneration in expanded CAG repeat diseases such as Huntington's disease. Despite intensive efforts, the mechanism of amyloid toxicity remains unknown. As a novel approach to probing polyQ toxicity, we investigate here how
Karunakar, Kar +4 more
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SWI/SNF senses carbon starvation with a pH-sensitive low-complexity sequence
eLife, 2022 It is increasingly appreciated that intracellular pH changes are important biological signals. This motivates the elucidation of molecular mechanisms of pH sensing.
J Ignacio Gutierrez +7 more
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