Fig. 2: Progerin expression in Hutchinson-Gilford progeria syndrome (HGPS) minipigs.

a Design of primers for PCR amplification of progerin mRNA. F forward primer, R reverse primer (which spans from progerin exon 12 to the 3′ end of exon 11 across the in-frame 150-nucleotide deletion produced by the alternative splicing caused by the LMNA c.1824C > T mutation). b Representative agarose gels showing PCR detection of progerin mRNA in wild-type (WT) and HGPS minipig tissues (each lane corresponds to a different pig). Skin fibroblasts from HGPS patients and healthy controls were used as positive and negative controls, respectively. First lane, DNA ladder. c Comparison of the C-terminal amino-acid sequences of human progerin and HGPS minipig progerin, which was predicted with ExPASy software from the DNA sequence of the gel-purified 482-bp PCR product specific to HGPS pigs (cf. b). d Representative immunoblots for lamin A/C and progerin protein in WT and HGPS minipig heart and aorta (heart: 60 µg protein/lane, aorta: 30 µg protein/lane; each lane corresponds to a different pig). Fibroblasts from WT and HGPS mice (Lmna^G609G) were used as progerin expression-negative and -positive controls, respectively. GADPH was used as loading control