Results 101 to 110 of about 16,368 (183)

Cell Death in Neurodegenerative Diseases: Molecular Mechanisms and Therapeutic Targets

MedComm, Volume 7, Issue 9, September 2026.
Regulated cell death pathways, including apoptosis, necroptosis, pyroptosis, ferroptosis, and autophagy‐dependent cell death, interact with mitochondrial dysfunction, proteostasis failure, lysosomal stress, glial remodeling, and neuroinflammation across major neurodegenerative diseases.
Tianjiao Li, Qian Zhang, Yichen Wu, Ruixue Huang   +3 more
wiley   +1 more source

Clinical Characteristics of C9ORF72-Linked Frontotemporal Lobar Degeneration

Dementia and Geriatric Cognitive Disorders Extra, 2013
Background: The most common genetic cause of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) has been linked to a hexanucleotide repeat expansion in the C9ORF72 gene.
Anna-Lotta Kaivorinne, Michaela K. Bode, Liisa Paavola, Hannu Tuominen, Mika Kallio, Alan E. Renton, Bryan J. Traynor, Virpi Moilanen, Anne M. Remes   +8 more
doaj   +1 more source

Muscle‐Specific Kinase Signaling and Its Therapeutic Potential

Muscle &Nerve, Volume 74, Issue S1, Page S65-S75, September 2026.
ABSTRACT The function of the neuromuscular junction (NMJ) is compromised in many neuromuscular diseases (NMDs) such as autoimmune or congenital myasthenia gravis (MG), amyotrophic lateral sclerosis (ALS), spinal muscular atrophy (SMA), and muscular dystrophies.
Stine Marie Jensen, Dana L. E. Vergoossen, Maartje G. Huijbers   +2 more
wiley   +1 more source

Integrated In Silico Discovery of Thymoquinone Analogs Targeting the Keap1–Nrf2 Pathway for Amyotrophic Lateral Sclerosis Therapy

ChemistryOpen, Volume 15, Issue 9, September 2026.
Integrated in silico discovery of thymoquinone analogs as potent Keap1 inhibitors for ALS therapy. Using validated QSAR, docking, and 200 ns MD simulations, we identified brain‐penetrant leads (CHEMBL3416163, CHEMBL4636830, and CHEMBL221598) with binding affinities up to fourfold stronger than thymoquinone, offering a targeted strategy to restore redox
Jabir C. Nalicho, Petro E. Mabeyo, Andrew S. Paluch, Lucas Paul   +3 more
wiley   +1 more source

Cell-type specific differences in promoter activity of the ALS-linked C9orf72 mouse ortholog

Scientific Reports, 2017
A hexanucleotide repeat expansion in the C9orf72 gene is the most common cause of inherited forms of the neurodegenerative disease amyotrophic lateral sclerosis (ALS).
Abraham J. Langseth, Juhyun Kim, Janet E. Ugolino, Yajas Shah, Ho-Yon Hwang, Jiou Wang, Dwight E. Bergles, Solange P. Brown   +7 more
doaj   +1 more source

Mapping the Young‐Onset Dementia Research in the Asia‐Pacific Region: A Scoping Review

Asia-Pacific Psychiatry, Volume 18, Issue 3, September 2026.
ABSTRACT Young‐onset dementia (YOD), with symptom onset before 65, is an area of increasing public health importance. YOD research in Asia‐Pacific remains under‐represented in the global YOD research landscape. This scoping review aimed to comprehensively map the existing YOD literature from Asia‐Pacific and provide an overview of the research topics ...
Gia Tan, Gary Cheung, Samantha M. Loi, Etuini Ma'u, Campbell Le Heron, Monica Cations, Nick Garrett, Megan Eustace, So Young Moon, Eun‐Joo Kim, Kok Pin Ng, Adeline S. L. Ng, Huali Wang, Isabelle Burke, Kenny Ong, Brigid Ryan   +15 more
wiley   +1 more source

Promotion of asthenozoospermia by C9orf72 through suppression of spermatogonia activity via fructose metabolism and mitophagy

Open Medicine
To investigate the involvement of C9orf72 in asthenozoospermia and its effects on spermatogonial energy metabolism and mitophagy.
Lu Hui, Xu Dongchuan, Zhao Liqiang, Ruan Hailing, Wang Anguo, Li Yejuan, Lu Weiying   +6 more
doaj   +1 more source

Proteomic profile of CSF obtained at the time of diagnosis determines amyotrophic lateral sclerosis progression and survival: CXCL7 levels in disease prognosis and survival

Brain Pathology, Volume 36, Issue 5, September 2026.
Untargeted multiomic profiling of cerebrospinal fluid reveals that proteomic, but not lipidomic, signatures robustly distinguish ALS patients from controls and stratify individuals by survival, highlighting marked molecular differences between short survival and long survival disease.
Sergio Roca‐Pereira, Yaiza López‐Sampere, Pol Mengod‐Soler, Mario Peña‐Fonteboa, Carla Marco, Alejandro Caravaca‐Puchades, Raúl Domínguez, Juan Francisco Vázquez‐Costa, Enrique Santamaría, Joaquín Fernández‐Irigoyen, María J. Colomina, Isabel León Moreno, Antonio Martínez Yélamos, Sergio Martínez Yélamos, Gabriel Santpere, Elia Obis, Manuel Portero‐Otín, Gerard Piñol‐Ripoll, Mónica Povedano, Pol Andrés‐Benito   +19 more
wiley   +1 more source

Loss of C9orf72 increases TFEB in the nucleus.

, 2016
A) Western analysis of TFEB levels after knockdown of C9orf72 in HEK293T cells. HEK293T were transfected with GFP-tagged TFEB and C9orf72 shRNA or control shRNA and then the lysates were analyzed using an antibody to GFP.
Justin Chu (732152), Yon Ju Ji (3359057), Janet Ugolino (3359054), Jiou Wang (267956), Karen Conchina (3359060), Akhilesh Pandey (14293), Raja Sekhar Nirujogi (841149), Anne Hamacher-Brady (177723), Nathan R. Brady (51959)   +8 more
core   +1 more source

Associations between TMEM106B C‐terminal fragment aggregation, age, and TDP‐43 or tau pathology

Brain Pathology, Volume 36, Issue 5, September 2026.
TMEM106B C‐terminal fragment (CTF) aggregation represents an age‐associated, common, diffuse phenomenon emerging after midlife with a weak association with TDP‐43 or tau pathology. These findings suggest that TMEM106B fibrillization may define a distinct axis of protein aggregation in the aging human brain. Abstract Transmembrane protein 106B (TMEM106B)
Albert Acewicz, Sylwia Tarka, Michał Grzegorczyk, Radosław Rzepliński, Teresa Wierzba‐Bobrowicz, Tomasz Stępień   +5 more
wiley   +1 more source