Results 31 to 40 of about 73,691 (271)

Predicting disease specific spinal motor neurons and glia in sporadic ALS

Neurobiology of Disease, 2019
Increasing evidence shows that neuroinflammation mediated by activated glia and infiltrated immune cells is involved in the pathogenesis of sporadic amyotrophic lateral sclerosis (sALS).
Fabien Dachet, Jiangou Liu, John Ravits, Fei Song   +3 more
doaj   +1 more source

Scaling proprioceptor gene transcription by retrograde NT3 signaling [PDF]

, 2011
The assembly of neuronal circuits depends critically on the sequential activation of transcriptional programs in defined neuronal sub-populations. In the spinal cord, retrograde signaling interactions from the periphery have been shown to be essential ...
Lee, Jun
core   +1 more source

A novel acylaminoimidazole derivative, WN1316, alleviates disease progression via suppression of glial inflammation in ALS mouse model. [PDF]

PLoS ONE, 2014
Amyotrophic lateral sclerosis (ALS) is an adult-onset motor neuron degenerative disease. Given that oxidative stress and resulting chronic neuronal inflammation are thought to be central pathogenic, anti-oxidative agents and modulators of neuronal ...
Kazunori Tanaka, Takuya Kanno, Yoshiko Yanagisawa, Kaori Yasutake, Satoshi Inoue, Noriaki Hirayama, Joh-E Ikeda   +6 more
doaj   +1 more source

Defining the optimal dose and therapeutic window in SMA with respiratory distress type I model mice, FVB/NJ-Ighmpb2 nmd-2J

Molecular Therapy: Methods & Clinical Development, 2021
Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is an autosomal recessive disorder that develops in infancy and arises from mutation of the immunoglobulin helicase μ-binding protein 2 (IGHMBP2) gene.
Monir Shababi, Caley E. Smith, Sara M. Ricardez Hernandez, Jose Marquez, Zayd Al Rawi, Eric Villalón, K. David Farris, Mona O. Garro-Kacher, Christian L. Lorson   +8 more
doaj   +1 more source

Does the survival motor neuron copy number variation play a role in the onset and severity of sporadic amyotrophic lateral sclerosis in Malians?

eNeurologicalSci, 2016
Introduction: Spinal muscular atrophy (SMA) and sporadic amyotrophic lateral sclerosis (SALS) are both motor neuron disorders. SMA results from the deletion of the survival motor neuron (SMN) 1 gene.
Modibo Sangare, Ilo Dicko, Cheick Oumar Guinto, Adama Sissoko, Kekouta Dembele, Youlouza Coulibaly, Siaka Y. Coulibaly, Guida Landoure, Abdallah Diallo, Mamadou Dolo, Housseini Dolo, Boubacar Maiga, Moussa Traore, Mamadou Karembe, Kadiatou Traore, Amadou Toure, Mariam Sylla, Arouna Togora, Souleymane Coulibaly, Sékou Fantamady Traore, Brant Hendrickson, Katherine Bricceno, Alice B. Schindler, Angela Kokkinis, Katherine G. Meilleur, Hammadoun Ali Sangho, Brehima Diakite, Yaya Kassogue, Yaya Ibrahim Coulibaly, Barrington Burnett, Youssoufa Maiga, Seydou Doumbia, Kenneth H. Fischbeck   +32 more
doaj   +1 more source

Candidate proteins, metabolites and transcripts in the Biomarkers for Spinal Muscular Atrophy (BforSMA) clinical study. [PDF]

PLoS ONE, 2012
Spinal Muscular Atrophy (SMA) is a neurodegenerative motor neuron disorder resulting from a homozygous mutation of the survival of motor neuron 1 (SMN1) gene. The gene product, SMN protein, functions in RNA biosynthesis in all tissues.
Richard S Finkel, Thomas O Crawford, Kathryn J Swoboda, Petra Kaufmann, Peter Juhasz, Xiaohong Li, Yu Guo, Rebecca H Li, Felicia Trachtenberg, Suzanne J Forrest, Dione T Kobayashi, Karen S Chen, Cynthia L Joyce, Thomas Plasterer, Pilot Study of Biomarkers for Spinal Muscular Atrophy Trial Group   +14 more
doaj   +1 more source

Distinct and common functions of mTORC1 and mTORC2 in Purkinje cells [PDF]

, 2015
In mammalian cells, the serine/threonine protein kinase mTOR (mammalian target of rapamycin) is present in two complexes, called mTORC1 and mTORC2. While several of the components are common to both complexes, raptor and rictor are only associated with ...
Angliker, Nico
core   +1 more source

A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice [PDF]

, 2005
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative condition characterized by motoneuron degeneration and muscle paralysis. Although the precise pathogenesis of ALS remains unclear, mutations in Cu/Zn superoxide dismutase (SOD1) account for
Dairin Kieran, Martin, J, Joanne Martin, Schiavo, G, Giampietro Schiavo (107907), Dick, JRT, Greensmith, L., Joanne Martin (11260388), Bohnert, S, Hafezparast, M., Elizabeth M.C. Fisher, Fisher, EMC, Linda Greensmith, Stephanie Bohnert (16066082), Martin, J., Majid Hafezparast (4461484), Fisher, E.M.C., James R T Dick (16066085), Majid Hafezparast, Greensmith, L, Linda Greensmith (252146), Giampietro Schiavo, Kieran, D., Bohnert, S., Stephanie Bohnert, James R.T. Dick, Dairin Kieran (7953794), Dick, J.R.T., Kieran, D, Hafezparast, M, Schiavo, G., Elizabeth M C Fisher (16062626)   +31 more
core   +1 more source

ATH-1105, a small-molecule positive modulator of the neurotrophic HGF system, is neuroprotective, preserves neuromotor function, and extends survival in preclinical models of ALS

Frontiers in Neuroscience
IntroductionAmyotrophic lateral sclerosis (ALS), a progressive and fatal neurodegenerative disorder, primarily affects the motor neurons of the brain and spinal cord.
Andrée-Anne Berthiaume, Sherif M. Reda, Kayla N. Kleist, Sharay E. Setti, Wei Wu, Jewel L. Johnston, Robert W. Taylor, Liana R. Stein, Hans J. Moebius, Kevin J. Church   +9 more
doaj   +1 more source

The SMN protein is a key regulator of nuclear architecture in differentiating neuroblastoma cells

, 2009
The cell nucleus contains two closely related structures, Cajal bodies (CBs) and gems. CBs are the first site of accumulation of newly assembled splicing snRNPs (small nuclear ribonucleoproteins) following their import into the nucleus, before they form ...
Nicholas P. Kinnear, Lisa Oram, Julie Burza, Clelland, Allyson K., Oram, Lisa, Allyson K. Clelland, Judith E. Sleeman, Kinnear, Nicholas P., Sleeman, Judith Elizabeth, Burza, Julie   +9 more
core   +1 more source