A novel peripherin isoform generated by alternative translation is required for normal filament network formation.

摘要: J. Neurochem. (2008) 104, 1663–1673. Abstract Peripherin is a type III neuronal intermediate filament protein detected within the intraneuronal inclusions characteristic of amyotrophic lateral sclerosis. The constitutively expressed peripherin isoform encoded by all nine exons human and mouse genes to generate species ∼58 kDa on sodium dodecyl sulfate–polyacrylamide gels. Expression this isoform, termed Per-58, generates network in transfected SW13 vim cells. On immunoblots cell lysates derived from these cells, we have consistently observed second ∼45 kDa. In study, show that novel generated through use an in-frame downstream initiation codon. This designated Per-45, co-expressed together with Per-58 and, thus, constitutive both mouse. Using mutational analysis, Per-45 required for normal formation, absence leading irregular filamentous structures. We further expression nervous system characterized tissue-specific Per-58 : Per-45 ratios. Taken together, results identify processing requirements indicate hitherto unrecognized role formation intra-isoform associations.