Results 71 to 80 of about 159,037 (298)

Diversity and complexity in neural organoids

FEBS Letters, EarlyView.
Neural organoid research aims to expand genetic diversity on one side and increase tissue complexity on the other. Chimeroids integrate multiple donor genomes within single organoids. Self‐organising multi‐identity organoids, exogenous cell seeding, or enforced assembly of region‐specific organoids contribute to tissue complexity.
Ilaria Chiaradia, Madeline A. Lancaster
wiley   +1 more source

SMN-deficient cells exhibit increased ribosomal DNA damage

Life Science Alliance, 2022
SMN deficiency leads to elevated ribosomal DNA damage that results in impaired ribosomal RNA synthesis and translation and is accompanied by a motor neuron-specific down-regulation of DDX21 protein.
Evangelia Karyka, Nelly Berrueta Ramirez, Christopher P Webster, Paolo M Marchi, Emily J Graves, Vinay K Godena, Lara Marrone, Anushka Bhargava, Swagat Ray, Ke Ning, Hannah Crane, Guillaume M Hautbergue, Sherif F El-Khamisy, Mimoun Azzouz   +13 more
doaj   +1 more source

Expression of a pathogenic mutation of SOD1 sensitizes aprataxin-deficient cells and mice to oxidative stress and triggers hallmarks of premature ageing [PDF]

, 2014
Aprataxin (APTX) deficiency causes progressive cerebellar degeneration, ataxia and oculomotor apraxia in man. Cell free assays and crystal structure studies demonstrate a role for APTX in resolving 5'-adenylated nucleic acid breaks, however, APTX ...
Bode, David, Carroll, Jean, Chiang, Shih-Chieh, El-Khamisy, Sherif F, Greensmith, Linda, Hafezparast, Majid, Kalmar, Bernadett, Mckinnon, Peter J, Page, Tristan K W, Thorpe, Julian R   +9 more
core   +1 more source

Linking neurogenesis, oligodendrogenesis, and myelination defects to neurodevelopmental disruption in primary mitochondrial disorders

FEBS Letters, EarlyView.
Mitochondrial remodeling shapes neural and glial lineage progression by matching metabolic supply with demand. Elevated OXPHOS supports differentiation and myelin formation, while myelin compaction lowers mitochondrial dependence, revealing mitochondria as key drivers of developmental energy adaptation.
Sahitya Ranjan Biswas, Porter L. Tomsick, Alicia M. Pickrell, Paul D. Morton   +3 more
wiley   +1 more source

Abnormal motoneuron migration, differentiation, and axon outgrowth in spinal muscular atrophy [PDF]

, 2008
The role of heterotopic (migratory) motoneurons (HMN) in the pathogenesis of spinal muscular atrophy (SMA) is still controversial. We examined the occurrence and amount of HMN in spinal cord tissue from eight children with SMA (six with SMA-I and two ...
Barišić, Nina, Hof, Patrick R., Islam, Atiqul, Jovanov Milošević, Nataša, Krušlin, Božo, Lucassen, Paul J., Mladinov, Mihovil, Pajtak, Alen, Sertić, Jadranka, Šešo Šimić, Đurđica, Šimić, Goran   +10 more
core   +1 more source

Potential therapeutic targeting of BKCa channels in glioblastoma treatment

Molecular Oncology, EarlyView.
This review summarizes current insights into the role of BKCa and mitoBKCa channels in glioblastoma biology, their potential classification as oncochannels, and the emerging pharmacological strategies targeting these channels, emphasizing the translational challenges in developing BKCa‐directed therapies for glioblastoma treatment.
Kamila Maliszewska‐Olejniczak, Karolina Pytlak, Sandra Jaworowska, Bogusz Kulawiak, Piotr Bednarczyk   +4 more
wiley   +1 more source

EGFR inhibitor erlotinib delays disease progression but does not extend survival in the SOD1 mouse model of ALS. [PDF]

PLoS ONE, 2013
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that causes progressive paralysis due to motor neuron death. Several lines of published evidence suggested that inhibition of epidermal growth factor receptor (EGFR) signaling might
Claire E Le Pichon, Sara L Dominguez, Hilda Solanoy, Hai Ngu, Nicholas Lewin-Koh, Mark Chen, Jeffrey Eastham-Anderson, Ryan Watts, Kimberly Scearce-Levie   +8 more
doaj   +1 more source

The glia response after peripheral nerve injury: A comparison between Schwann cells and olfactory ensheathing cells and their uses for neural regenerative therapies [PDF]

, 2017
The peripheral nervous system (PNS) exhibits a much larger capacity for regeneration than the central nervous system (CNS). One reason for this difference is the difference in glial cell types between the two systems.
Barton, Matthew J., Clarke, Mary, Ekberg, Jenny, St. John, James, Wright, Alison   +4 more
core   +2 more sources

Edited GluR2, a gatekeeper for motor neurone survival? [PDF]

BioEssays, 2008
AbstractAmyotrophic lateral sclerosis (ALS) is a progressive degenerative disorder of motor neurones. Although the genetic basis of familial forms of ALS has been well explored, the molecular basis of sporadic ALS is less well understood. Recent evidence has linked sporadic ALS with the failure to edit key residues in ionotropic glutamate receptors ...
Buckingham, S. D., Kwak, S., Jones, A. K., Blackshaw, S. E., Sattelle, David B.   +4 more
openaire   +3 more sources

Exploiting metabolic adaptations to overcome dabrafenib treatment resistance in melanoma cells

Molecular Oncology, EarlyView.
We show that dabrafenib‐resistant melanoma cells undergo mitochondrial remodeling, leading to elevated respiration and ROS production balanced by stronger antioxidant defenses. This altered redox state promotes survival despite mitochondrial damage but renders resistant cells highly vulnerable to ROS‐inducing compounds such as PEITC, highlighting redox
Silvia Eller, Susanne Ebner, Carmen Haselrieder, Julia K. Günther, Astrid Drasche, Sophie Strich, Chiara Volani, Andrea Medici, Aleksandar Nikolajevic, Alex Deltedesco, Johannes E. Sigmund, Michael J. Blumer, Martin Hermann, Johanna Vanacker, Gerald Brandacher, Eduard Stefan, Omar Torres‐Quesada, Jakob Troppmair   +17 more
wiley   +1 more source