Results 91 to 100 of about 19,301 (228)

E3 ligase Praja1 mediates ubiquitination and degradation of microtubule‐associated protein tau

The FEBS Journal, EarlyView.
E3 ligase Praja1, but not its paralogue Praja2, recognizes and ubiquitinates tau protein for proteasomal degradation. This newly identified function of Praja1‐mediated tau degradation suggests its role in protein quality control, which may provide insights into the pathogenesis of tauopathies.
Shiho Aoki, Wataru Onodera, Akihiko Takashima, Kotaro Kawasaki, Kazuki Imadegawa, Hikaru Kurahashi, Mizuho Oishi, Toru Asahi, Yoshiyuki Soeda   +8 more
wiley   +1 more source

Effects of overexpression of Huntingtin proteins on mitochondrial integrity [PDF]

Human Molecular Genetics, 2008
Huntington's disease (HD) is caused by an expansion of a CAG trinucleotide sequence that encodes a polyglutamine tract in the huntingtin (Htt) protein. Expansion of the polyglutamine tract above 35 repeats causes disease, with the age of onset inversely related to the degree of expansion above this number.
Hongmin, Wang, Precious J, Lim, Mariusz, Karbowski, Mervyn J, Monteiro   +3 more
openaire   +2 more sources

Redox environment modulates aggregation of ataxin‐3 in vitro — Implications for drug screening of cysteine‐rich proteins

The FEBS Journal, EarlyView.
Redox environment modulates in vitro aggregation of Ataxin‐3, the protein implicated in spinocerebellar ataxia type 3. Reducing conditions stabilize native monomers and prevent aggregation, whereas oxidative conditions promote the formation of non‐native conformers and disulfide‐linked oligomers within the Josephin domain (JD).
Martyna Podlasiak, Martina Sollazzo, Elisa Monaca, Raffaele Sabbatella, Maria Agnese Morando, Oleg Chertkov, Laura Puglisi, Martina Mascellino, Anna Fricano, Sandra Macedo‐Ribeiro, Caterina Alfano   +10 more
wiley   +1 more source

The Role of Post-translational Modifications on the Energy Landscape of Huntingtin N-Terminus

Frontiers in Molecular Biosciences, 2019
Huntington disease is a neurodegenerative disease characterized by a polymorphic tract of polyglutamine repeats in exon 1 of the huntingtin protein, which is thought to be responsible for protein aggregation and neuronal death. The polyglutamine tract is
Havva Yalinca, Charlotte Julie Caroline Gehin, Vladimiras Oleinikovas, Hilal A. Lashuel, Francesco Luigi Gervasio, Francesco Luigi Gervasio, Annalisa Pastore   +6 more
doaj   +1 more source

Proteostasis of organelles in aging and disease

The FEBS Journal, EarlyView.
Cells rely on regulated proteostasis mechanisms to keep their internal compartments functioning properly. When these mechanisms fail, damaged proteins accumulate, disrupting organelles, such as the nucleus, mitochondria, endoplasmic reticulum, Golgi, and lysosomes, as well as membraneless organelles, such as stress granules, processing bodies, the ...
Yara Nabawi, Cansu Doğan, Dunja Petrović, Gülce Perçin, Seda Koyuncu, David Vilchez   +5 more
wiley   +1 more source

The causative role of amyloidosis in the cardiac complications of Alzheimer's disease: a comprehensive systematic review

The Journal of Physiology, EarlyView.
Abstract figure legend Schematic illustration of the bidirectional causative link between cerebral amyloid‐beta (Aβ) angiopathy and cardiovascular disease in Alzheimer's disease (AD). Common cardiovascular risk factors like microvascular thrombosis, diabetes, atrial fibrillation, hypertension and atherosclerosis lead to cerebral hypoperfusion and ...
Samuel Parker, Andrew F. James, Svetlana Mastitskaya   +2 more
wiley   +1 more source

Mutant Huntingtin Forms in Vivo Complexes with Distinct Context-Dependent Conformations of the Polyglutamine Segment

Neurobiology of Disease, 1999
Huntington's disease (HD) is caused by an expanded glutamine tract, which confers a novel aggregation-promoting property on the 350-kDa huntingtin protein.
Francesca Persichetti, Flavia Trettel, C.Chris Huang, Cornel Fraefel, H.T.Marc Timmers, James F. Gusella, Marcy E. MacDonald   +6 more
doaj   +1 more source

AOSNP‐ADAPTR resource level‐based recommendations on practical diagnostic strategies for WHO CNS5 adult‐type diffuse gliomas

Brain Pathology, Volume 36, Issue 2, March 2026.
ADAPTR recommendations for Adult‐type Diffuse Gliomas in Resource‐restrained settings. Abstract The fifth edition of the WHO classification of CNS Tumors (WHO CNS5) has revised the diagnostic and grading criteria for Adult‐type Diffuse Gliomas (ADGs) by integrating molecular parameters with histologic features.
Vani Santosh, Chitra Sarkar, Shilpa Rao, Ho‐Keung Ng, Junji Shibahara, Maysa Al‐Hussaini, Michael E. Buckland, Sung‐Hye Park, Tarik Tihan, Pieter Wesseling, David N. Louis, Takashi Komori   +11 more
wiley   +1 more source

Reduced Levels of Proteasome Products in a Mouse Striatal Cell Model of Huntington's Disease.

PLoS ONE, 2015
Huntington's disease is the result of a long polyglutamine tract in the gene encoding huntingtin protein, which in turn causes a large number of cellular changes and ultimately results in neurodegeneration of striatal neurons. Although many theories have
Sayani Dasgupta, Michael A Fishman, Hana Mahallati, Leandro M Castro, Alexandre K Tashima, Emer S Ferro, Lloyd D Fricker   +6 more
doaj   +1 more source

New Insights on Heat Shock Proteins as Regulators of Reactive Oxygen Species Across Various Stressors in Diseases

Cell Biochemistry and Function, Volume 44, Issue 2, February 2026.
ABSTRACT Living beings are persistently challenged by stress. Stress can be induced by internal stressors and external stressors. External stressors, including radiation, heat, heavy metals, nutritional imbalances, infections, and psychological stress, can induce protein denaturation, leading to misfolded or aggregated proteins.
Paka Sravan Kumar, Triveni Kodi, Adarsh Gopinathan, Bharath Harohalli Byregowda, Krishnadas Nandakumar, Anoop Kishore   +5 more
wiley   +1 more source