Other proteins that contain this repeating unit, known as a "neurexin motif", include those members of the NCP (neurexin IV/Caspr/paranodin) family that have been implicated in neuron-glial and glial-glial interactions [15], Flamingo cadherins [16], and crumbs (Crb) homologue proteins [17].
The suggestion that these fatty acids are then intra-cellularly internalised within lysosomes [ 78] correlates with intriguing observations from E. coloratus induced pathology, where increases in the size and numbers of lysosomes within the neuronal tissue of guinea pigs were implicated in neuron lysis and cerebral damage [ 79].
PAPC, FLRT3 and RND1 are all expressed in other tissues that all express various classical cadherins, including neurons, and are implicated in neuronal morphology and activity [17], [21], [22], and it will be important to examine their functional interactions in the regulation of cell adhesion in these other tissues.
Nurr1 is a gene that is critical for the survival and maintenance of dopaminergic neurons and has been implicated in dopaminergic neuron related disorders.
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].
There was a significant 1.03 fold Log2 up-regulation of the Fused in Sarcoma transcript (Fus), which has been implicated in motor neuron pathology in amyotrophic lateral sclerosis (ALS), and the protein has been shown to interact with Smn [ 18, 59].
Abnormalities in protein homeostasis have been implicated in motor neuron diseases (Nishimura et al. 2004; Petrucelli and Dawson 2004; Johnson et al. 2010; Deng et al. 2011; Rubino et al. 2012), but the impact of a dysfunctional UCHL1 on the health and stability of motor neurons, especially upper motor neurons, remains poorly understood.
