This contrasts with aSN being mainly presynaptic under normal circumstances. With respect to adverse effects on the neuronal cell in its entirety, it remains unclear whether pre- or post-synaptic changes are compromised first. Interestingly, muscles contained small angulated fibers reminiscent of neurogenic muscular atrophy . In addition, we found that neuromuscular synapses showed signs of presynaptic degeneration although less pronounced as reported previously in lines expressing haSN . a- Bungarotoxin staining patterns for postsynaptic acetylcholine receptors were not different between wt and Thy1-maSN mice , neither in soleus nor extensor digitorum longus muscles . Also, we detected little or no changes between wt and transgenic mice in presynaptic synaptophysin staining . In contrast, staining of presynaptic neurofilaments differed dramatically. The neuromuscular junctions in Thy1-maSN mice showed thinning or absence of presynaptic neurofilament staining . In summary, neuromuscular junctions in Thy1-maSN mice showed degeneration that was independent of muscle fiber type and similar, as reported for mice expressing haSN transgene . Double labeling for ubiquitin and P-Ser129aSN was Enlarged mitochondria are a sign of cells trying to compensate for energy 439081-18-2 deficits, reflecting local increases in the need for energy, vacuolization and/or loss of inner-outer membrane integrity of thein paraffin sections of neurons in regions such as the cortex, where only very few cells stained for ubiquitin and additionally in regions with pronounced ubiquitin pathology such as brainstem, colliculus and spinal cord . This revealed an extraordinary staining pattern, in particular, in processes. As shown, P-Ser129aSN and ubiquitin immunopositive stretches in processes alternate and did not overlap . In contrast, in cell somata, the distribution patterns of P-Ser129aSN and ubiquitin were strikingly similar and overlaped to a high extent . Enlarged mitochondria are a sign of cells trying to compensate for energy deficits, reflecting local increases in the need for energy, vacuolization and/or loss of inner-outer membrane integrity of the mitochondria.