Supplementary Materials

Supplementary Material for:

Laminopathies disrupt epigenomic developmental programs and cell fate

Jelena Perovanovic, Stefania Dell'Orso, Viola F. Gnochi, Jyoti K. Jaiswal, Vittorio Sartorelli, Corinne Vigouroux, Kamel Mamchaoui, Vincent Mouly, Gisèle Bonne, Eric P. Hoffman*

*Corresponding author. E-mail: ehoffman{at}

Published 20 April 2016, Sci. Transl. Med. 8, 335ra58 (2016)
DOI: 10.1126/scitranslmed.aad4991

This PDF file includes:

  • Fig. S1. Transfection of DamLMNA constructs into HEK293T cells.
  • Fig. S2. LADs show enrichment for heterochromatin and are negatively correlated with euchromatin.
  • Fig. S3. Myogenin locus localized closer to the nuclear periphery in EDMD myotubes.
  • Fig. S4. Myogenic in vitro differentiation of MyoD-converted EDMD patient fibroblasts (p.H222P LMNA) into myoblasts.
  • Fig. S5. DNA methylation analysis of lamin A/C [wild-type, EDMD (LMNA p.R453W), and FPLD (LMNA p.R482W] transduced human skeletal myogenic cells.
  • Fig. S6. Genome-wide analysis of H3K9me3 peaks in wild-type and emerin-null (emd) differentiating mouse myoblasts shows similar peak distributions.
  • Fig. S7. Sox2 protein levels show abnormal up-regulation in emerin-null murine cells and EDMD patients harboring LMNA p.H222P mutation.
  • Fig. S8. EDMD LMNA mutation significantly inhibits myogenin expression in human myogenic cells infected with LMNA constructs.
  • Table S1. Genome-wide DamID-seq analysis of three lamin A proteins (wild-type, p.R453W LMNA causing EDMD, and p.R482W LMNA causing FPLD).
  • Table S2. Genes showing mRNA induction at myoblast-myotube transition (ENCODE).
  • Table S3. DNA methylation data of normal and EDMD patient MyoD-converted myoblasts normalized to d1.
  • Table S4. Human myogenic cells infected with EDMD LMNA variant show differential DNA methylation levels on myogenic and cell cycle genes.
  • Table S5. Heterochromatin peaks (H3K9me3) showing decreased enrichment in emerin-null cell during myogenic transition (d0 to d1).
  • Table S6. Characteristics of the patient muscle biopsies used for mRNA profiling.
  • Table S7. ChIP-seq targets show up-regulation in muscle biopsies from EDMD patients.
  • Table S8. Primer sequences used for qRT-PCR and ChIP-qPCR experiments.

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