Ontology highlight
ABSTRACT:
SUBMITTER: Kedia N
PROVIDER: S-EPMC6708308 | biostudies-literature | 2019 Aug
REPOSITORIES: biostudies-literature
Kedia Niraja N Arhzaouy Khalid K Pittman Sara K SK Sun Yuanzi Y Batchelor Mark M Weihl Conrad C CC Bieschke Jan J
Proceedings of the National Academy of Sciences of the United States of America 20190801 34
Desmin-associated myofibrillar myopathy (MFM) has pathologic similarities to neurodegeneration-associated protein aggregate diseases. Desmin is an abundant muscle-specific intermediate filament, and disease mutations lead to its aggregation in cells, animals, and patients. We reasoned that similar to neurodegeneration-associated proteins, desmin itself may form amyloid. Desmin peptides corresponding to putative amyloidogenic regions formed seeding-competent amyloid fibrils. Amyloid formation was ...[more]