Increased accumulation of transferrin by motor neurons of the mouse mutant progressive motor neuronopathy ( pmn/pmn )

摘要: It has been suggested that iron-carrying transferrin exerts growth-factor-like influences on motor neurons. I have evaluated the distribution of proteins related to intracerebral iron-homeostasis in mouse mutant progressive neuronopathy (pmn/pmn); an autosomal recessive with caudo-cranial neuron degeneration. A higher immunoreactivity and receptor neurons thepmn/pmn compared normal mice was demonstrated. Ferritin not observed mutant. Transferrin receptors were absent from axons neuromuscular junctions, indicating entry blood-borne, liver-derived (‘liver transferrin’) into due uptake subsequent retrograde axonal transport unspecific. Due selective presence neuronal somata, a more likely mode by receptor-mediated brain-derived (‘brain at soma. This study provides data accumulation expression diseased adds further insights PNS.