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End of a busy day of PhD defenses. Congratulations to Dr Raffaella Parlato from my lab! And also to Dr Vasista Adupa from the Onck lab! Two #polyQ containing theses and defenses. :-)

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UniQure and FDA No Longer in Alignment on Approval Pathway for AMT-130 – HDBuzz

New article @hdbuzzfeed.bsky.social about AMT-130: “UniQure and FDA No Longer in Alignment on Approval Pathway for AMT-130” en.hdbuzz.net/uniqure-and-... #huntingtonsdisease #polyQ

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Pathogenesis of polyglutamine diseases: Piecing together a complex molecular puzzle | Journal of Experimental Medicine | Rockefeller University Press This comprehensive review by Villavicencio Gonzalez and Zoghbi integrates established and emerging insights into the pathogenic mechanisms of polyglutamine

New review article “Pathogenesis of polyglutamine diseases: Piecing together a complex molecular puzzle” by Esmeralda Villavicencio Gonzalez and Huda Zoghbi in the Journal of Experimental Medicine: doi.org/10.1084/jem.... #polyQ #huntingtonsdisease #ataxia

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Exogenous Huntingtin-Exon1 Aggregates Exhibit Distinct Levels of Toxicity to Caenorhabditis elegans Many neurodegenerative diseases, including Alzheimer’s (AD), Parkinson’s (PD), and Huntington’s disease (HD), are associated with proteinaceous deposits in the brain comprising amyloid. The aggregatio...

Exogenous Huntingtin-Exon1 Aggregates Exhibit Distinct Levels of Toxicity to Caenorhabditis elegans | ACS Chemical Neuroscience pubs.acs.org/doi/abs/10.1... Interesting new paper on fundamental mechanisms in #huntingtonsdisease by Justin Legleiter’s team at the Univ of Nevada #polyQ

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Ashwani Thakur

Ashwani Thakur

Ashwani Thakur presents his talk

Ashwani Thakur presents his talk

It was great to have Prof Ashwani Thakur, from IIT Kanpur, present his talk about #amyloid protein deposits in human disease and in plants (where they are ‘functional’). From #polyQ to plant seeds!

Catch him at the amyloid meeting in Hamburg. @landaulabcssb.bsky.social @cssbhamburg.bsky.social

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Probing How Anti‐huntingtin Antibodies Bind the Fibrillar Fuzzy Coat Using Solid‐State NMR Antibodies are widely used to recognize, detect, and target aggregated and misfolded amyloid-forming proteins, including those associated with HD. Studying such interactions on a structural level is ...

… I will be there, to discuss our recent paper on #ssNMR studies of antibodies binding to #polyQ protein fibrils ( #huntingtonsdisease ). (ref. dx.doi.org/10.1002/chem... ) …

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Probing How Anti‐huntingtin Antibodies Bind the Fibrillar Fuzzy Coat Using Solid‐State NMR Antibodies are widely used to recognize, detect, and target aggregated and misfolded amyloid-forming proteins, including those associated with HD. Studying such interactions on a structural level is ....

.. in #huntingtonsdisease but may also find effective use in studies of other aggregating #amyloid proteins, from #alzheimers to #parkinsons and beyond.

Read it all at the journal: doi.org/10.1002/chem... (where it is part of the “Hot Topic: amyloids” collection.

#nmrchat #polyQ #ssNMR

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.. Like in #Alzheimers or #Parkinsons, HD patient brains contain aggregated clumps of protein, which in HD feature parts of the huntingtin protein. This #polyQ protein forms amyloid-like fibrils, which are often detectable by the use of antibodies that selectively recognize exposed epitopes. ..

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Probing How Anti‐huntingtin Antibodies Bind the Fibrillar Fuzzy Coat Using Solid‐State NMR Antibodies are widely used to recognize, detect, and target aggregated and misfolded amyloid-forming proteins, including those associated with HD. Studying such interactions on a structural level is ....

Online: doi.org/10.1002/chem... “Probing How Anti-huntingtin Antibodies Bind the Fibrillar Fuzzy Coat Using Solid-State #NMR” - continuing our research on #huntingtonsdisease and in particular the aggregated #polyQ proteins involved. Here we looked at antibodies binding to the latter. #nmrchat

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.. These studies were done with our #polyQ huntingtin exon 1 proteins, but presumably similar things can happen with other #amyloid proteins. Moreover, a deeper mechanistic understanding of what happened here is still work in process…

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The mutation is at first glance a bit unusual, but it is actually far from unique: a repeating sequence of CAG codons has been expanded in HD patients. This translates to huntingtin (HTT) proteins featuring an expanded #polyglutamine (or #polyQ) segment. HD patients have >35 glutamines. (2/..)

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Integrative determination of atomic structure of mutant huntingtin exon 1 fibrils implicated in Huntington disease - Nature Communications An integrated structural biology approach encompassing solid-state NMR and molecular dynamics simulations was implemented to obtain an atomic view of the ordered polyglutamine core and fuzzy coat of t...

Now available at the journal: Integrative determination of atomic structure of mutant huntingtin exon 1 fibrils implicated in Huntington disease

doi.org/10.1038/s414...

#polyq #amyloid

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Sage Journals: Discover world-class research Subscription and open access journals from Sage, the world's leading independent academic publisher.

Interesting new paper in the Journal of Huntington Disease: An analysis of the native structure of #huntingtin (and other #polyQ proteins), informed my recent discoveries about the structural biology of huntingtin. #huntingtonsdisease #ataxia

doi.org/10.1177/1879...

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Solid-state nuclear magnetic resonance in the structural study of polyglutamine aggregation The aggregation of proteins into amyloid-like fibrils is seen in many neurodegenerative diseases. Recent years have seen much progress in our understanding of these misfolded protein inclusions, thank...

Amidst the stress of moving the #NMR instrument, a bit of happy news: our review article on #ssNMR studies of #polyQ protein aggregates is now online in Biochemical Society Transactions: doi.org/10.1042/BST2... #nmrchat #amyloid

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Yay! It is now online: our latest paper on the use #NMR spectroscopy to look at #amyloid formation by #polyQ peptides outfitted with a backbone modification, which allows photochemical control over beta-hairpin formation. Together with the groups of Szymanski and Feringa in #Groningen.

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