Results 41 to 50 of about 19,301 (228)

Aberrant Huntingtin Interacting Protein 1 in Lymphoid Malignancies [PDF]

Cancer Research, 2007
Abstract Huntingtin interacting protein 1 (HIP1) is an inositol lipid, clathrin, and actin binding protein that is overexpressed in a variety of epithelial malignancies. Here, we report for the first time that HIP1 is elevated in non–Hodgkin's and Hodgkin's lymphomas and that patients with lymphoid malignancies frequently had anti ...
Sarah V, Bradley, Mitchell R, Smith, Teresa S, Hyun, Peter C, Lucas, Lina, Li, Danielle, Antonuk, Indira, Joshi, Fang, Jin, Theodora S, Ross   +8 more
openaire   +2 more sources

Alterations in mRNA 3′ UTR Isoform Abundance Accompany Gene Expression Changes in Human Huntington’s Disease Brains

Cell Reports, 2017
The huntingtin gene has two mRNA isoforms that differ in their 3′ UTR length. The relationship of these isoforms with Huntington’s disease is not established.
Lindsay Romo, Ami Ashar-Patel, Edith Pfister, Neil Aronin   +3 more
doaj   +1 more source

Robust Cell-Free Expression of Sub-Pathological and Pathological Huntingtin Exon-1 for NMR Studies. General Approaches for the Isotopic Labeling of Low-Complexity Proteins

Biomolecules, 2020
The high-resolution structural study of huntingtin exon-1 (HttEx1) has long been hampered by its intrinsic properties. In addition to being prone to aggregate, HttEx1 contains low-complexity regions (LCRs) and is intrinsically disordered, ruling out ...
Anna Morató, Carlos A. Elena-Real, Matija Popovic, Aurélie Fournet, Karen Zhang, Frédéric Allemand, Nathalie Sibille, Annika Urbanek, Pau Bernadó   +8 more
doaj   +1 more source

A huntingtin peptide inhibits polyQ-huntingtin associated defects. [PDF]

PLoS ONE, 2013
Huntington's disease (HD) is caused by the abnormal expansion of the polyglutamine tract in the human Huntingtin protein (polyQ-hHtt). Although this mutation behaves dominantly, huntingtin loss of function also contributes to HD pathogenesis.
Yoan Arribat, Nathalie Bonneaud, Yasmina Talmat-Amar, Sophie Layalle, Marie-Laure Parmentier, Florence Maschat   +5 more
doaj   +1 more source

Altered microRNA expression in animal models of Huntington’s disease and potential therapeutic strategies

Neural Regeneration Research, 2021
A review of recent animal models of Huntington’s disease showed many microRNAs had altered expression levels in the striatum and cerebral cortex, and which were mostly downregulated.
Bridget Martinez, Philip V Peplow
doaj   +1 more source

Expression of expanded polyglutamine targets profilin for degradation and alters actin dynamics

Neurobiology of Disease, 2008
Huntington's disease is caused by polyglutamine expansion in the huntingtin protein. Huntingtin directly interacts with profilin, a major actin monomer sequestering protein and a key integrator of signals leading to actin polymerization.
Barrington G. Burnett, Jaime Andrews, Srikanth Ranganathan, Kenneth H. Fischbeck, Nicholas A. Di Prospero   +4 more
doaj   +1 more source

Nuclear and cytoplasmic huntingtin inclusions exhibit distinct biochemical composition, interactome and ultrastructural properties

Nature Communications, 2021
The mechanisms underlying Huntingtin protein (Htt) aggregation are not fully understood. Here the authors perform a detailed investigation of the ultrastructural and biochemical properties of huntingtin cytoplasmic and nuclear inclusions, and reveal that
Nathan Riguet, Anne-Laure Mahul-Mellier, Niran Maharjan, Johannes Burtscher, Marie Croisier, Graham Knott, Janna Hastings, Alice Patin, Veronika Reiterer, Hesso Farhan, Sergey Nasarov, Hilal A. Lashuel   +11 more
doaj   +1 more source

Huntingtin’s spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function

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, Raquel Epand, Alexander Leitner, Tae-Yang Jung, Baehyun Shin, Roy Jung, Alejandro Lloret, Randy Singh Atwal, Hyeongseok Lee, Jong-Min Lee, Ruedi Aebersold, Hans Hebert, Ji-Joon Song, Ihn Sik Seong   +13 more
doaj   +1 more source

HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration. [PDF]

PLoS Biology, 2013
Histone deacetylase (HDAC) 4 is a transcriptional repressor that contains a glutamine-rich domain. We hypothesised that it may be involved in the molecular pathogenesis of Huntington's disease (HD), a protein-folding neurodegenerative disorder caused by ...
Michal Mielcarek, Christian Landles, Andreas Weiss, Amyaouch Bradaia, Tamara Seredenina, Linda Inuabasi, Georgina F Osborne, Kristian Wadel, Chrystelle Touller, Rachel Butler, Janette Robertson, Sophie A Franklin, Donna L Smith, Larry Park, Paul A Marks, Erich E Wanker, Eric N Olson, Ruth Luthi-Carter, Herman van der Putten, Vahri Beaumont, Gillian P Bates   +20 more
doaj   +1 more source

scyllo-Inositol Promotes Robust Mutant Huntingtin Protein Degradation [PDF]

Journal of Biological Chemistry, 2014
Huntington disease is characterized by neuronal aggregates and inclusions containing polyglutamine-expanded huntingtin protein and peptide fragments (polyQ-Htt). We have used an established cell-based assay employing a PC12 cell line overexpressing truncated exon 1 of Htt with a 103-residue polyQ expansion that yields polyQ-Htt aggregates to ...
Aaron Y, Lai, Cynthia P, Lan, Salwa, Hasan, Mary E, Brown, Joanne, McLaurin   +4 more
openaire   +2 more sources