Invertebrate models of lamin diseases

Figures & data

Figure 1. The in vitro structure of the C. elegans lamin filament: (A) Wildtype Ce-lamin paracrystalline arrays. (B) Wildtype Ce-lamin filaments. (C) Ce-lamin paracrystalline arrays of the progeria Q159K mutation. (D) Ce-lamin filament-like structure of the Q159K mutation. The repeat unit of the filament is different. (E) Paracrystalline arrays of Ce-lamin with the EDMD L535P mutation. While filaments apparently look like wildtype filaments (not shown) they order in the Paracrystalline arrays is lost. (F) The repeat units in Ce-lamin filament containing the EDMD DeltaK46 mutation are 34 and 14 nm compared to 21 and 27 nm in the assembled wildtype protein [45, 55]. Scale bars are 50 nm for panels A-E.

Figure 2. Transmission electron microscopy images reveals muscle abnormalities in animals expressing GFP:ΔK46. Longitudinal (long) (A) and transverse (trans) sections of wildtype animals or animals expressing GFP:ΔK46. Data is taken from Fig, 8 in [43].

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Bank EM, Ben-Harush K, Feinstein N, et al. Structural and physiological phenotypes of disease-linked lamin mutations in C. elegans. J Struc Biol. 2011;177:106–12.

 PubMed Web of Science ®Google Scholar

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