Supplementary Materials

Supplementary Material for:

Splicing-Directed Therapy in a New Mouse Model of Human Accelerated Aging

Fernando G. Osorio, Claire L. Navarro, Juan Cadiñanos, Isabel C. López-Mejía, Pedro M. Quirós, Catherine Bartoli, José Rivera, Jamal Tazi, Gabriela Guzmán, Ignacio Varela, Danielle Depetris, Félix de Carlos, Juan Cobo, Vicente Andrés, Annachiara De Sandre-Giovannoli, José M. P. Freije, Nicolas Lévy, Carlos López-Otín*

*To whom correspondence should be addressed. E-mail: clo{at}

Published 26 October 2011, Sci. Transl. Med. 3, 106ra107 (2011)
DOI: 10.1126/scitranslmed.3002847

This PDF file includes:

  • Fig. S1. Genomic sequencing of Lmna exon 11 in Lmna+/+, LmnaG609G/+, and LmnaG609G/G609G mice.
  • Fig. S2. Western (immuno) blot analysis of human control fibroblasts (AG10803), human HGPS fibroblasts (AG01972c), and tissues from Lmna+/+, LmnaG609G/+, and LmnaG609G/G609G mice.
  • Fig. S3. Phenotypic characterization of LmnaLCS/LCS mice.
  • Fig. S4. Organ size evaluation in 3-month-old LmnaG609G/G609G versus Lmna+/+ mice.
  • Fig. S5. µCT analysis of bone alterations in LmnaG609G/G609G mice.
  • Fig. S6. Cardiovascular phenotype of LmnaG609G/G609G mice.
  • Fig. S7. Enrichment score plots from GSEA-extracted representative pathways containing genes enriched in LmnaG609G/G609G mice samples.
  • Fig. S8. Enrichment score plots of GSEA-extracted representative pathways containing genes enriched in Lmna+/+ mice samples.
  • Fig. S9. Nuclear envelope architecture analyzed in an HGPS fibroblast cell line (AG01972c) treated with HsEx10 and HsEx11 morpholinos.
  • Fig. S10. RT-PCR analysis in tissues from Lmna+/+, untreated LmnaG609G/G609G, and MmEx10-11–treated LmnaG609G/G609G and LmnaG609G/+ mice.
  • Fig. S11. Western (immuno) blot analysis of lamin A/C in tissues from treated and untreated LmnaG609G/G609G mice.
  • Fig. S12. Phenotypic characterization of MmEx10-11–treated LmnaG609G/G609G mice.
  • Table S1. Affymetrix Mouse Gene 1.0 ST probes showing the greatest increase or decrease (P < 0.005) in liver from LmnaG609G/G609G mutant mice.

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