Results 1 to 10 of about 19,992 (185)

Full-length huntingtin is palmitoylated at multiple sites and post-translationally myristoylated following caspase-cleavage

Frontiers in Physiology, 2023
Introduction: Huntington disease is an autosomal dominant neurodegenerative disorder which is caused by a CAG repeat expansion in the HTT gene that codes for an elongated polyglutamine tract in the huntingtin (HTT) protein.
Fanny L. Lemarié, Shaun S. Sanders, Yen Nguyen, Dale D. O. Martin, Michael R. Hayden   +4 more
doaj   +1 more source

IKBKB reduces huntingtin aggregation by phosphorylating serine 13 via a non-canonical IKK pathway

Life Science Alliance, 2023
Early exposure of RA induced altered expressions of Wnt-related genes and subsequent osteogenic differentiation in embryonic mouse craniofacial prominences in a spatiotemporal-dependent manner.
Cristina Cariulo, Paola Martufi, Margherita Verani, Leticia Toledo-Sherman, Ramee Lee, Celia Dominguez, Lara Petricca, Andrea Caricasole   +7 more
doaj   +1 more source

Huntingtin is critical both pre- and postsynaptically for long-term learning-related synaptic plasticity in Aplysia. [PDF]

PLoS ONE, 2014
Patients with Huntington's disease exhibit memory and cognitive deficits many years before manifesting motor disturbances. Similarly, several studies have shown that deficits in long-term synaptic plasticity, a cellular basis of memory formation and ...
Yun-Beom Choi, Beena M Kadakkuzha, Xin-An Liu, Komolitdin Akhmedov, Eric R Kandel, Sathyanarayanan V Puthanveettil   +5 more
doaj   +1 more source

Comparative molecular dynamics simulations of pathogenic and non-pathogenic huntingtin protein monomers and dimers

Frontiers in Molecular Biosciences, 2023
Polyglutamine expansion at the N-terminus of the huntingtin protein exon 1 (Htt-ex1) is closely associated with a number of neurodegenerative diseases, which result from the aggregation of the increased polyQ repeat.
Mohammed Khaled, Birgit Strodel, Birgit Strodel, Abdallah Sayyed-Ahmad   +3 more
doaj   +1 more source

Significant Association Between Huntingtin Gene Mutation and Prevalence of Hopelessness, Depression and Anxiety Symptoms

Acta Medica Lituanica, 2021
Background: In Huntington’s disease psychiatric symptoms may manifest prior to motor dysfunction. Such symptoms negatively impact people’s quality of life and can worsen the course of the primary disease.
Adelė Butėnaitė, Robertas Strumila, Aistė Lengvenytė, Indrė Kotryna Pakutkaitė, Aušra Morkūnienė, Aušra Matulevičienė, Edgaras Dlugauskas, Algirdas Utkus   +7 more
doaj   +1 more source

Tissue Transglutaminase Selectively Modifies Proteins Associated with Truncated Mutant Huntingtin in Intact Cells

Neurobiology of Disease, 2001
The cause of Huntington's disease (HD) is a pathological expansion of the polyglutamine domain within the N-terminal region of huntingtin. Neuronal intranuclear inclusions and cytoplasmic aggregates composed of the mutant huntingtin within certain ...
Wanjoo Chun, Mathieu Lesort, Janusz Tucholski, Peter W. Faber, Marcy E. MacDonald, Christopher A. Ross, Gail V.W. Johnson   +6 more
doaj   +1 more source

Investigating Crosstalk Among PTMs Provides Novel Insight Into the Structural Basis Underlying the Differential Effects of Nt17 PTMs on Mutant Httex1 Aggregation

Frontiers in Molecular Biosciences, 2021
Post-translational modifications (PTMs) within the first 17 amino acids (Nt17) of the Huntingtin protein (Htt) have been shown to inhibit the aggregation and attenuate the toxicity of mutant Htt proteins in vitro and in various models of Huntington’s ...
Anass Chiki, Zhidian Zhang, Zhidian Zhang, Kolla Rajasekhar, Luciano A. Abriata, Iman Rostami, Iman Rostami, Lucien F. Krapp, Driss Boudeffa, Matteo Dal Peraro, Hilal A. Lashuel   +10 more
doaj   +1 more source

Huntingtin is required for ER-to-Golgi transport and for secretory vesicle fusion at the plasma membrane

Disease Models & Mechanisms, 2014
Huntingtin is a large membrane-associated scaffolding protein that associates with endocytic and exocytic vesicles and modulates their trafficking along cytoskeletal tracks. Although the progression of Huntington’s disease is linked to toxic accumulation
Hemma Brandstaetter, Antonina J. Kruppa, Folma Buss   +2 more
doaj   +1 more source

Inducible mutant huntingtin expression in HN10 cells reproduces Huntington's disease-like neuronal dysfunction

Molecular Neurodegeneration, 2009
Background Expansion of a polyglutamine repeat at the amino-terminus of huntingtin is the probable cause for Huntington's disease, a lethal progressive autosomal-dominant neurodegenerative disorders characterized by impaired motor performance and severe ...
Paganetti Paolo, Roscic Ana, Weiss Andreas   +2 more
doaj   +1 more source

Huntingtin Regulates Mammary Stem Cell Division and Differentiation

Stem Cell Reports, 2014
Little is known about the mechanisms of mitotic spindle orientation during mammary gland morphogenesis. Here, we report the presence of huntingtin, the protein mutated in Huntington’s disease, in mouse mammary basal and luminal cells throughout ...
Salah Elias, Morgane S. Thion, Hua Yu, Cristovao Marques Sousa, Charlène Lasgi, Xavier Morin, Sandrine Humbert   +6 more
doaj   +1 more source