Results 111 to 120 of about 19,027,943 (182)

Mesenchymal Stem Cell‐Based Therapy for Cerebellar Ataxia: From Bench to Bedside

CNS Neuroscience &Therapeutics, Volume 32, Issue 7, July 2026.
Allogeneic hMSCs transplanted across LPS, Ara‐C, and SCA2 cerebellar ataxia (CA) models suppress neuroinflammation and restore the neurotrophin axis, collectively preserving Purkinje cell integrity. These preclinical findings are being translated clinically, from a first‐in‐human case report to an ongoing Phase II/III randomized trial (NCT02540655 ...
Kyoungho Suk, Ho‐Won Lee, Sang Ryong Kim   +2 more
wiley   +1 more source

Early‐Onset Oral Squamous Cell Carcinoma: Emerging Biological Insights, Risk Factors and Clinical Implications

Journal of Oral Pathology &Medicine, Volume 55, Issue 6, Page 629-641, July 2026.
ABSTRACT Background Early‐onset oral squamous cell carcinoma (EO‐OSCC), commonly defined as occurring in individuals under 50 years of age, is increasingly recognized as a potentially distinct clinical subset with differences in exposure patterns and tumor biology compared with conventional oral squamous cell carcinoma (OSCC).
Gennaro Musella, Cristina D'Antonio, Federica Canfora, Michele D. Mignogna, Valentino Vellone, Vito Carlo Alberto Caponio, Amerigo Giudice, Alessandro Villa, Daniela Adamo   +8 more
wiley   +1 more source

Multiomics Profiling During Autoimmune Demyelination Highlights a Complex Regulatory Role for Ataxin‐1 in B Cells

Annals of the New York Academy of Sciences, Volume 1561, Issue 1, July 2026.
Ataxin‐1 has been recently implicated in B cell regulation in the context of multiple sclerosis pathology. In this study, we used a multiomics pipeline to profile the B cell transcriptome, epigenome, and proteome in response to encephalitogenic challenges.
Jonathan J. Carver, Rachael R. Denbrock, Kristy M. Lau, Tonya N. Zeczycki, Changhong Yin, Weihua Huang, Alessandro Didonna   +6 more
wiley   +1 more source

Quinazolinone and Phthalazinone Inhibitors of the HDAC6/Ubiquitin Protein–Protein Interaction

ChemBioChem, Volume 27, Issue 12, 26 June 2026.
Here, we report the validation and expansion of small‐molecule inhibitors targeting the HDAC6 ZnF‐UBD pocket. Following molecular modeling, hits were synthesized, and candidate compounds were tested in fluorescence polarization of ubiquitin C‐terminal peptide displacement.
Sydney Gordon, Jordi C. J. Hintzen, Sebastian Dilones, Brockton Keen, Callie E. W. Crawford, George M. Burslem   +5 more
wiley   +1 more source

Amyloid precursor-like protein 2 cleavage contributes to neuronal intranuclear inclusions and cytotoxicity in spinocerebellar ataxia-7 (SCA7)

Neurobiology of Disease, 2011
In spinocerebellar ataxia-7 (SCA7), a polyglutamine (polyQ) expansion in the ataxin-7 protein leads to the formation of neuronal intranuclear inclusions (NIIs) and neurodegeneration.
Junko Takahashi-Fujigasaki, Tilo Breidert, Hiroto Fujigasaki, Charles Duyckaerts, Jacques H. Camonis, Alexis Brice, Anne-Sophie Lebre   +6 more
doaj   +1 more source

The blood-brain barrier is disrupted in Machado-Joseph disease/spinocerebellar ataxia type 3: evidence from transgenic mice and human post-mortem samples

Acta Neuropathologica Communications, 2020
Blood-brain barrier (BBB) disruption is a common feature in neurodegenerative diseases. However, BBB integrity has not been assessed in spinocerebellar ataxias (SCAs) such as Machado-Joseph disease/SCA type 3 (MJD/SCA3), a genetic disorder, triggered by ...
Diana Duarte Lobo, Rui Jorge Nobre, Catarina Oliveira Miranda, Dina Pereira, João Castelhano, José Sereno, Arnulf Koeppen, Miguel Castelo-Branco, Luís Pereira de Almeida   +8 more
doaj   +1 more source

The Effect of Protein Tagging on Aggregation and Phase Separation

Journal of Cellular Biochemistry, Volume 127, Issue 6, June 2026.
ABSTRACT Protein tags are widely used for purification, solubilization, detection, and imaging, yet they can substantially alter protein self‐assembly. This interference is particularly significant for intrinsically disordered proteins and low‐complexity domains, whose aggregation and phase separation are mediated by weak multivalent interactions that ...
Harunobu Saito, Kenji Sugase
wiley   +1 more source

Ataxin-3 phosphorylation protects neurons [PDF]

J Cell Biol, 2016
![Figure][1] Expanded ataxin-3 causes an extensive loss of neurons (brown) in rat brains (left). But the amount of neurodegeneration is reduced when serine 12 is mutated to aspartate (right).
europepmc   +3 more sources

Adipose-Derived Mesenchymal Stem Cells Improve Motor Function and Reduce Neuroinflammation and Mutant Ataxin-3 Protein Levels in SCA3 Mice.

International Journal of Stem Cells
The CAG expansion in the ataxin-3 (ATXN3) protein is the underlying cause of Spinocerebellar Ataxia Type 3 (SCA3), a polyglutamine disease. The aggregation of mutant ATXN3 protein is hypothesized to contribute to neuronal dysfunction, neurodegeneration ...
Kuan-Ming Lu, W. Ong, ChingHuai Ko, Ling-Mei Wang, A. Ting, Hsiu-Yu Lai   +5 more
semanticscholar   +1 more source

Allosteric regulation of deubiquitylase activity through ubiquitination

Frontiers in Molecular Biosciences, 2015
Ataxin-3, the protein responsible for spinocerebellar ataxia type-3, is a cysteine protease that specifically cleaves poly-ubiquitin chains and participates in the ubiquitin proteasome pathway.
Serena eFaggiano, Rajesh P. Menon, Geoff P. Kelly, Sokol V. Todi, K. Matthew Scaglione, Petr V. Konarev, Petr V. Konarev, Dmitri I. Svergun, Henry L. Paulson, Annalisa ePastore   +9 more
doaj   +1 more source