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Protein structural and mechanistic basis of progeroid laminopathies
Author(s) -
Marcelot Agathe,
Worman Howard J.,
ZinnJustin Sophie
Publication year - 2021
Publication title -
the febs journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.981
H-Index - 204
eISSN - 1742-4658
pISSN - 1742-464X
DOI - 10.1111/febs.15526
Subject(s) - lamin , progeria , lmna , nuclear lamina , missense mutation , biology , premature aging , genetics , mutation , intermediate filament , microbiology and biotechnology , gene , nuclear protein , cytoskeleton , cell , transcription factor
Progeroid laminopathies are characterized by the premature appearance of certain signs of physiological aging in a subset of tissues. They are caused by mutations in genes coding for A‐type lamins and associated proteins. These mutations cause either structural defects or altered protein interactions. Characterizing the molecular changes detected in patients through structural biology studies could facilitate the design of therapeutic strategies to treat these rare but severe diseases.