Fig. 2.

Cellular effects of the wobbler mutation. The schematic drawing show a lower motor neuron connected to skeletal muscle cells and associated with interneurons and glial cells, such as astrocytes, microglial cells, oligodendrocytes and Schwann cells. The effects on the different cells, motor neuron degeneration muscle atrophy, astrogliosis, microgliosis and the loss of GABAergic interneurons are indicated. The effects of the dysfunction of cellular processes are given with numbers: (1) the formation of APP- and Rab7-positive vacuoles, (2) neurofilament aggregations, (3) impaired axonal transport, (4) further ubiquitin-positive protein aggregates and mitochondrial dysfunction (5)