Lmna-c.C1827T

Fig.1 Detection of Lmna expression in colon by RT-PCR. 

Homozygous, heterozygous and wild type: one band at 288 bp with primers F1/R1(mLmna) and one band at 123 bp with primers F/R(mGapdh). 

Abbr. M, DNA marker; HO, homozygous; HE, heterozygous; WT, wild type.

Fig.2 The sequencing results are as expected.

Fig.3 Detection of  Lmna expression in Lmna-c.C1827T mice by WB. (Red Arrow indicates expected Progerin band)

Abbr. M, marker; WT, wild type; HO, Homozygous; HE, heterozygous; PC, positive control, Hela cells.

Fig.4 The body weight curve of WT C57BL/6 and HO Lmna(G609G) mice. (female, n=1)

Abbr. WT, wild type；HO, Homozygous

Fig.5 Representative CT scan images of WT C57BL/6 and HO Lmna (G609G) mice of 6.6 weeks. (female, n=1)

Fig.6 Representative CT scan images of WT C57BL/6 and HO Lmna (G609G) mice of 10.6 weeks. (female, n=1)

Fig.7 Representative CT scan images of WT C57BL/6 and HO Lmna (G609G) mice of 14.7 weeks. (female, n=1)

Fig.8 Relevant evaluation metrics of WT C57BL/6 and HO Lmna (G609G) mice at 6.6, 10.6, and 14.7 weeks (w) of age (female, n=1). A) Kyphosis Index (KI), B) Tibia length ), C) Skull length, D) Vertebral column length.

Fig.9 Body size of WT C57BL/6 and HO Lmna (G609G) mice. (female, n=1)

Fig.10 Representative H&E staining images of Skin/subcutaneous adipose tissue in WT and HO Lmna (G609G) mice. (female, n=1)

Fig.11 Representative H&E staining images of Aorta in WT and HO Lmna (G609G) mice. (femal, n=1) 

Fig.12 Representative H&E staining images of  Skeletal muscle in WT and HO Lmna (G609G) mice. (female, n=1)

Fig.13 Representative H&E staining images of heart in WT and HO Lmna (G609G) mice. (female, n=1)

Fig.14 Body Weight and body Weight change of Lmna(G609G) mice (start at 6 weeks old). 

Fig.15 Survival analysis, with the death date recorded based on a 20% weight loss threshold (start at 6 weeks old).

Fig.16 Detection of LMNA expression in 16-wk-old (day 71) Lmna(G609G) mice by WB (Red Arrow indicates expected Progerin band). 

Abbr. M, marker. G1, WT, female; G2, WT, male; G3, HO Lmna(G609G), female; G4, HO Lmna(G609G), male; G5, HO Lmna(G609G), male, Zokinvy(lonafarnib), p.o., 80mg/kg

Fig.17 Representative micro CT images of 16-week-old mice.

Abbr.  G1, WT, female; G2, WT, male; G3, HO Lmna(G609G), female; G4, HO Lmna(G609G), male; G5, HO Lmna(G609G), male, Zokinvy(lonafarnib), p.o., 80mg/kg

Fig.18 The Photograph and Weight of Heart, Spleen and Thymus in 16-week-old mice.

Fig.19 Detection of fasting glucose level at day 71. 

Fig.20 Health condition description. Numerical scoring system used to describe the health, with detailed scoring at 98 days shown in the right table.