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The BG have been implicated in motor control and cognitive switching tasks; in particular, it is believed that the BG function as a controller for motor tasks by selectively disinhibiting appropriate portions of the thalamus and hence activating, via a feedback loop, cortical regions.
Examples include mutations in tubulin folding co-factor E [7], the dynactin complex [8] [9] and the microtubule severing protein, spastin [10], which have all been implicated in motor neuron degeneration.
In the past decade, VEGF has been implicated in motor neuron degeneration in mice.
p75NTR has been implicated in motor neuron death in humans and in SOD1G93A mice.
In particular OLIG1 and NKX6-2 have been implicated in motor neuron differentiation early in development [ 56- 58].
The caudate nucleus has been implicated in motor planning, especially in demanding conditions [ 42] which were also absent during the run.
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There are also reports that central sensory pathways can be implicated in motor neuron disease (Hamada et al., 2007; Mochizuki et al., 2011).
Soluble misfolded Cu/Zn superoxide dismutase (SOD1) is implicated in motor neuron death in amyotrophic lateral sclerosis (ALS); however, the relative toxicities of the various non-native species formed by SOD1 as it misfolds and aggregates are unknown.
Interestingly, Calreticulin has been implicated in regulating motor neuron pathology in a related motor neuron disease (amyotrophic lateral sclerosis; ALS) [ 43], suggesting that, alongside its potential to act as a molecular biomarker for SMA, further investigations into its possible contribution to the pathogenesis of SMA are warranted.
These copper chaperones have since been implicated in certain motor neuron disorders and diseases of copper metabolism, such as Wilson's and Menkes diseases.
The TG has also been implicated in coordinating motor patterns, generating rhythmic movements in flies and crickets and gregarious behavior in locusts [37] [39].
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