Results 81 to 90 of about 130,299 (263)

Restoration of full-length SMN promoted by adenoviral vectors expressing RNA antisense oligonucleotides embedded in U7 snRNAs. [PDF]

PLoS ONE, 2009
BACKGROUND:Spinal Muscular Atrophy (SMA) is an autosomal recessive disease that leads to specific loss of motor neurons. It is caused by deletions or mutations of the survival of motor neuron 1 gene (SMN1). The remaining copy of the gene, SMN2, generates
Till Geib, Klemens J Hertel
doaj   +1 more source

Modulating the Folding Landscape of Superoxide Dismutase 1 with Targeted Molecular Binders [PDF]

, 2018
Amyotrophic lateral sclerosis, or Lou Gehrig's disease, is characterized by motor neuron death with average survival times of 2 ‐ 5 years. One cause of this disease is the misfolding of superoxide dismutase 1 (SOD1), a protein whose stability and ...
Atsavapranee, Beatriz, Bunck, David N., Heath, James R., Museth, Anna K., VanderVelde, David G.   +4 more
core  

Sodium vanadate combined with l-ascorbic acid delays disease progression, enhances motor performance, and ameliorates muscle atrophy and weakness in mice with spinal muscular atrophy [PDF]

, 2013
BACKGROUND: Proximal spinal muscular atrophy (SMA), a neurodegenerative disorder that causes infant mortality, has no effective treatment. Sodium vanadate has shown potential for the treatment of SMA; however, vanadate-induced toxicity in vivo remains an
Chen-Hung Ting, Hsin-Lan Wen, Hsiu-Mei Hsieh-Li, Huei-Chun Liu, Hung Li, Li-Kai Tsai, Sue Lin-Chao   +6 more
core   +1 more source

Targeting TNBC: core–shell polycationic polyurea dendrimers with inherent anticancer activity

FEBS Open Bio, EarlyView.
Core–shell polycationic PURE dendrimers were tested in TNBC‐derived tumor models. Both formulations selectively targeted TNBC and effectively reduced tumor volume. PUREG4‐OEI48 suppressed tumor growth without detectable toxicity, whereas PUREG4‐OCEI24, despite showing efficacy, induced hepatic toxicity.
Adriana Cruz, Bruna Abreu, Cindy Mendes, Catarina Freitas‐Dias, Filipe Gonçalves, Fernanda Silva, José Ramalho, Saudade André, Vasco D. B. Bonifácio, Jacinta Serpa   +9 more
wiley   +1 more source

The drug adaptaquin blocks ATF4/CHOP-dependent pro-death Trib3 induction and protects in cellular and mouse models of Parkinson's disease

Neurobiology of Disease, 2020
Identifying disease-causing pathways and drugs that target them in Parkinson's disease (PD) has remained challenging. We uncovered a PD-relevant pathway in which the stress-regulated heterodimeric transcription complex CHOP/ATF4 induces the neuron ...
Pascaline Aimé, Saravanan S. Karuppagounder, Apeksha Rao, Yingxin Chen, Robert E. Burke, Rajiv R. Ratan, Lloyd A. Greene   +6 more
doaj   +1 more source

Neuron-Specific HuR-Deficient Mice Spontaneously Develop Motor Neuron Disease [PDF]

, 2018
Human Ag R (HuR) is an RNA binding protein in the ELAVL protein family. To study the neuron-specific function of HuR, we generated inducible, neuron-specific HuR-deficient mice of both sexes.
Bai, Ying, Chen, Xing, Hamilton, Thomas A., Herjan, Tomasz, Kokiko-Cochran, Olga N., Lamb, Bruce, Lee, Yu-Shang, Li, Xiao, Lin, Ching-Yi, Sun, Kevin, Zhou, Gao   +10 more
core   +1 more source

KLK7 overexpression promotes an aggressive phenotype and facilitates peritoneal dissemination in colorectal cancer cells

FEBS Open Bio, EarlyView.
KLK7, a tissue kallikrein‐related peptidase, is elevated in advanced colorectal cancer and associated with shorter survival. High KLK7 levels in ascites correlate with peritoneal metastasis. In mice, KLK7 overexpression increases metastasis. In vitro, KLK7 enhances cancer cell proliferation, migration, adhesion, and spheroid formation, driving ...
Yosr Z. Haffani, Tobias Dreyer, Meriem Naim, Rea Lo Dico, Natalia A. Ignatenko, Viktor Magdolen, Dalila Darmoul   +6 more
wiley   +1 more source

Defining the organizational structure of dopamine and muscarninic acetylcholine receptors [PDF]

, 2015
No abstract ...
Alvarez-Curto, Elisa, Caltabiano, Gianluigi Caltabiano, Liste, María José Varela, Marsango, Sara, Milligan, Graeme   +4 more
core   +1 more source

The role of lipid metabolism in neuronal senescence

FEBS Open Bio, EarlyView.
Disrupted lipid metabolism, through alterations in lipid species or lipid droplet accumulation, can drive neuronal senescence. However, lipid dyshomeostasis can also occur alongside neuronal senescence, further amplifying tissue damage. Delineating how lipid‐induced senescence emerges in neurons and glial cells, and how it contributes to ageing and ...
Dikaia Tsagkari, Eleftheria Panagiotidou, Nektarios Tavernarakis   +2 more
wiley   +1 more source

Exercise attenuates polyglutamine‐mediated neuromuscular degeneration in a mouse model of spinal and bulbar muscular atrophy

Journal of Cachexia, Sarcopenia and Muscle
Background Spinal and bulbar muscular atrophy (SBMA) is a hereditary neuromuscular disorder caused by the expansion of trinucleotide cytosine–adenine–guanine (CAG) repeats, which encodes a polyglutamine (polyQ) tract in the androgen receptor (AR) gene ...
Tomoki Hirunagi, Hideaki Nakatsuji, Kentaro Sahashi, Mikiyasu Yamamoto, Madoka Iida, Genki Tohnai, Naohide Kondo, Shinichiro Yamada, Ayuka Murakami, Seiya Noda, Hiroaki Adachi, Gen Sobue, Masahisa Katsuno   +12 more
doaj   +1 more source