Adducin at the Neuromuscular Junction in Amyotrophic Lateral Sclerosis: Hanging on for Dear Life.

摘要: The neurological dysfunction in amyotrophic lateral sclerosis (ALS)/motor neurone disease (MND) is associated with defective nerve-muscle contacts early the suggesting that perturbations of cell adhesion molecules (CAMs) linking pre- and post-synaptic components neuromuscular junction (NMJ) are involved. To search for candidate proteins implicated this degenerative process, researchers have studied Drosophila larval NMJ find cytoskeleton-associated protein, adducin, ideally placed to regulate synaptic contacts. By controlling levels proteins, adducin can de-stabilize Interestingly, elevated phosphorylated been reported ALS patients a mouse model disease. Adducin regulated by phosphorylation through protein kinase C (PKC), some isoforms which exhibit Ca(2+)-dependence, raising possibility changes intracellular Ca(2+) might alter PKC activation secondarily influence phosphorylation. Furthermore, has interactions alpha subunit Na(+)/K(+)-ATPase. Thus, may stability at so integrity ALS.