taiwanese sma mouse model  (Jackson Laboratory)

Journal: International Journal of Molecular Sciences

Article Title: Long-Term SMN - and Ncald -ASO Combinatorial Therapy in SMA Mice and NCALD -ASO Treatment in hiPSC-Derived Motor Neurons Show Protective Effects

doi: 10.3390/ijms24044198

Figure Lengend Snippet: Relevant information about hiPSC lines.

Article Snippet: The severely-affected Taiwanese SMA mouse model [FVB.Cg-Tg ( SMN2 )2Hung Smn1 tm1Hung/J, stock number 005058] [ ] was purchased from Jackson Laboratory (Bar Harbor, ME, USA).

Techniques: Sampling, Control, Plasmid Preparation

Journal: Life Science Alliance

Article Title: Short-duration splice promoting compound enables a tunable mouse model of spinal muscular atrophy

doi: 10.26508/lsa.202000889

Figure Lengend Snippet: Mice were injected s.c. with 1 mg/kg NVS-SM2 or vehicle starting PND 2 daily until PND 6. Mice were euthanized and tissues harvested on PND 7. (A, B, C) Human SMN protein levels were analyzed in brain (A), spinal cord (B), and muscle (C) tissues via immunoblotting. SMN protein was normalized to actin and tubulin. Each lane represents tissue from an individual mouse. SMA represents the severe 5058 SMA mice, which express the human SMN2 transgene, and Het mice are their littermates, expressing both human and mouse SMN.

Article Snippet: The less-used, slightly more severe “Li” or “Taiwanese” SMA mouse model (Jackson Labs; FVB.Cg-Smn1tm1HungTg(SMN2)2Hung/J.) also lacks murine Smn and expresses the human SMN2 transgene ( ).

Techniques: Injection, Western Blot, Expressing

Journal: Life Science Alliance

Article Title: Short-duration splice promoting compound enables a tunable mouse model of spinal muscular atrophy

doi: 10.26508/lsa.202000889

Figure Lengend Snippet: (A) Kaplan–Meier survival curves of severe 5058 SMA mice s.c. treated with vehicle (n = 7) or 0.1 (n = 4) and 1 mg/kg (n = 5) NVS-SM2 daily until PND 15 and then every other day until PND 30 (A). Mantel–Cox test was used to analyze survival differences between NVS-treated and SMA mice and P -values are presented in the legend. (B, C, D) Mice were monitored for body weights (B) and tail length (C, D). Data expressed as SEM.

Article Snippet: The less-used, slightly more severe “Li” or “Taiwanese” SMA mouse model (Jackson Labs; FVB.Cg-Smn1tm1HungTg(SMN2)2Hung/J.) also lacks murine Smn and expresses the human SMN2 transgene ( ).

Techniques:

Journal: Life Science Alliance

Article Title: Short-duration splice promoting compound enables a tunable mouse model of spinal muscular atrophy

doi: 10.26508/lsa.202000889

Figure Lengend Snippet: Severe 5058 SMA mice were treated on PND 2, PND 3 and PND 4 s.c. or orally (p.o.) with the indicated doses of NVS-SM2. (A) Kaplan–Meier survival curve of severe 5058 SMA mice s.c. treated with 0.1 (n = 6), 0.5 (n = 6), and 1 mg/kg (n = 6) NVS-SM2 or orally with 1 mg/kg NVS-SM2 (n = 4) and untreated SMA mice (n = 10). Mantel–Cox test was used to analyze survival differences between NVS-treated and SMA mice and P -values are presented in the legend. (B, C, D) Mice were monitored for body weights (B) and tail length (C) to determine the tail length: body weight ratio (D). (E) Pen test time of 0.5 mg/kg NVS-SM2–treated mice in comparison to Het mice (E). Data expressed as SEM.

Article Snippet: The less-used, slightly more severe “Li” or “Taiwanese” SMA mouse model (Jackson Labs; FVB.Cg-Smn1tm1HungTg(SMN2)2Hung/J.) also lacks murine Smn and expresses the human SMN2 transgene ( ).

Techniques: Comparison

Journal: Life Science Alliance

Article Title: Short-duration splice promoting compound enables a tunable mouse model of spinal muscular atrophy

doi: 10.26508/lsa.202000889

Figure Lengend Snippet: Severe SMA mice were treated on PND 2 and PND 3 with the indicated doses of NVS-SM2. (A) Kaplan–Meier survival curve of severe 5058 SMA mice treated s.c. with 0.1 mg/kg NVS-SM2 (n = 5) on PND 2 and PND 3 in comparison to untreated SMA mice (n = 6). Mantel–Cox test was used to analyze survival differences between NVS-treated and SMA mice and P -values are presented in the legend. (B) Mice were monitored for body weights. Data expressed as SEM.

Article Snippet: The less-used, slightly more severe “Li” or “Taiwanese” SMA mouse model (Jackson Labs; FVB.Cg-Smn1tm1HungTg(SMN2)2Hung/J.) also lacks murine Smn and expresses the human SMN2 transgene ( ).

Techniques: Comparison

Journal: Life Science Alliance

Article Title: Short-duration splice promoting compound enables a tunable mouse model of spinal muscular atrophy

doi: 10.26508/lsa.202000889

Figure Lengend Snippet: Animals were treated starting on PND 6 (A, n = 3) or PND 8 (B, n = 14) s.c. with 1 mg/kg NVS-SM2. (A, B) Kaplan–Meier survival curves. PND 8–treated SMA mice were further separated into non-survivors (n = 8) and survivors (n = 6) groups. Mantel–Cox test was used to analyze survival differences between NVS-treated and SMA mice and P -values are presented in the legend. (C, D) PND 8–treated severe 5058 SMA mice were monitored for body weights (C) and tail lengths (D). Data expressed as SEM.

Article Snippet: The less-used, slightly more severe “Li” or “Taiwanese” SMA mouse model (Jackson Labs; FVB.Cg-Smn1tm1HungTg(SMN2)2Hung/J.) also lacks murine Smn and expresses the human SMN2 transgene ( ).

Techniques:

Journal: Life Science Alliance

Article Title: Short-duration splice promoting compound enables a tunable mouse model of spinal muscular atrophy

doi: 10.26508/lsa.202000889

Figure Lengend Snippet: (A) Spleen weights of NVS-SM2–treated severe 5058 SMA mice at PND 110 in comparison to healthy Het control mice are not different (A). (B) Average body weight at PND 8 of the treated NVS-SM2 mice separated into the survivors and non-survivors groups are not different (B). Data expressed as SEM and analyzed using Student’s unpaired t test, a P -value of 0.05 was taken as significant. ns indicates nonsignificance.

Article Snippet: The less-used, slightly more severe “Li” or “Taiwanese” SMA mouse model (Jackson Labs; FVB.Cg-Smn1tm1HungTg(SMN2)2Hung/J.) also lacks murine Smn and expresses the human SMN2 transgene ( ).

Techniques: Comparison, Control

Journal: Life Science Alliance

Article Title: Short-duration splice promoting compound enables a tunable mouse model of spinal muscular atrophy

doi: 10.26508/lsa.202000889

Figure Lengend Snippet: (A, B, C, D) Immunoblot of human SMN and housekeeping proteins at PND 110 in Het (n = 4) and NVS-SMN2 PND 8–treated severe SMA 5058 mice (n = 5) in brain (A, B) and spinal cord (C, D). SMN protein was normalized to housekeeping proteins (B, D). Each lane represents tissue from an individual mouse. Data expressed as SEM and analyzed using Student's unpaired t test, a P -value of 0.05 was taken as significant.

Article Snippet: The less-used, slightly more severe “Li” or “Taiwanese” SMA mouse model (Jackson Labs; FVB.Cg-Smn1tm1HungTg(SMN2)2Hung/J.) also lacks murine Smn and expresses the human SMN2 transgene ( ).

Techniques: Western Blot