Profound alteration of the oxygen consumption rate (QO2) is present in the cervical spinal
cord (CS) of the wobbler mice aged 12 weeks (wr12). Early symptomatic mice at 4 weeks …

The Wobbler mouse, a mutant characterized by motoneuron degeneration in the cervical
spinal cord, has been used to test the efficacy of novel treatments for human motoneuron …

The wobbler mouse is one of the most useful models of motoneuron degeneration,
characterized by selective motoneuronal death in the cervical spinal cord. We carried out …

In the present study, we investigated whether cultured astrocytes derived from adult neural
precursor cells (NPCs) obtained from the subventricular zone (SVZ) of wobbler mice display …

Neurofilament (NF) aggregate formation within motor neurons is a pathological hallmark of
both the sporadic and familial forms of amyotrophic lateral sclerosis (ALS). The relationship …

Impaired axonal transport has been observed in patients with amyotrophic lateral sclerosis
(ALS) and in animal models, suggesting that transport proteins likely play a critical role in the …

We show here, by high resolution sodium dodecyl sulfate gel electrophoresis, that the
proportions of myosin heavy chain (MyHC) isoforms of mouse muscles are specifically …

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease. So far, no
cure exists, prompting studies in disease mechanisms to facilitate development of new …

The wobbler mouse is a model of selective motor neuron degeneration in the cervical spinal
cord. Comparing cervical and lumbar tracts of control and diseased mice at the early stage …

Usp8 is a deubiquitinating enzyme that works as regulator of endosomal trafficking and is
involved in cell proliferation.“In vivo” USP8 is predominantly expressed in the central …