Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS

BJ Turner, K Talbot - Progress in neurobiology, 2008 - Elsevier
Gain-of-function mutations in the Cu, Zn-superoxide dismutase (SOD1) gene are implicated
in progressive motor neuron death and paralysis in one form of inherited amyotrophic lateral …

Potential roles of gut microbiome and metabolites in modulating ALS in mice

E Blacher, S Bashiardes, H Shapiro, D Rothschild… - Nature, 2019 - nature.com
Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disorder, in which the
clinical manifestations may be influenced by genetic and unknown environmental factors …

Neurogenesis impairment: An early developmental defect in Down syndrome

F Stagni, A Giacomini, M Emili, S Guidi… - Free Radical Biology and …, 2018 - Elsevier
Down syndrome (DS) is characterized by brain hypotrophy and intellectual disability starting
from early life stages. Accumulating evidence shows that the phenotypic features of the DS …

[HTML][HTML] Brainstem pathology in amyotrophic lateral sclerosis and primary lateral sclerosis: a longitudinal neuroimaging study

P Bede, RH Chipika, E Finegan, SLH Shing… - NeuroImage: Clinical, 2019 - Elsevier
Background Brainstem pathology is a hallmark feature of ALS, yet most imaging studies
focus on cortical grey matter alterations and internal capsule white matter pathology …

[HTML][HTML] MRI in rodent models of brain disorders

A Denic, SI Macura, P Mishra, JD Gamez, M Rodriguez… - …, 2011 - Elsevier
Magnetic resonance imaging (MRI) is a well-established tool in clinical practice and
research on human neurological disorders. Translational MRI research utilizing rodent …

NAP (davunetide) modifies disease progression in a mouse model of severe neurodegeneration: protection against impairments in axonal transport

Y Jouroukhin, R Ostritsky, Y Assaf, G Pelled… - Neurobiology of …, 2013 - Elsevier
NAP (davunetide) is a novel neuroprotective compound with mechanism of action that
appears to involve microtubule (MT) stabilization and repair. To evaluate, for the first time …

Hind limb muscle atrophy precedes cerebral neuronal degeneration in G93A-SOD1 mouse model of amyotrophic lateral sclerosis: a longitudinal MRI study

S Marcuzzo, I Zucca, A Mastropietro, NK de Rosbo… - Experimental …, 2011 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a progressive, fatal, neurodegenerative disorder
caused by the degeneration of motor neurons in the CNS, which results in complete …

Is it possible to improve neurodevelopmental abnormalities in Down syndrome?

R Bartesaghi, S Guidi, E Ciani - 2011 - degruyter.com
Down syndrome (DS) is a genetic pathology caused by the triplication of human
chromosome 21. Although individuals with DS have various medical problems, intellectual …

Mental retardation and associated neurological dysfunctions in Down syndrome: a consequence of dysregulation in critical chromosome 21 genes and associated …

M Rachidi, C Lopes - European Journal of Paediatric Neurology, 2008 - Elsevier
Down syndrome (DS), affecting 1/700 live births, is the major genetic cause of mental
retardation (MR), a cognitive disorder with hard impact on public health. DS brain is …

Mental retardation in Down syndrome: from gene dosage imbalance to molecular and cellular mechanisms

M Rachidi, C Lopes - Neuroscience research, 2007 - Elsevier
Down syndrome (DS), the most frequent genetic disorder leading to mental retardation (MR),
is caused by three copies of human chromosome 21 (HC21). Trisomic and transgenic …