Figure - PMC

Skip to main content

An official website of the United States government

Here's how you know

Here's how you know

Official websites use .gov
A .gov website belongs to an official government organization in the United States.

Secure .gov websites use HTTPS
A lock ( ) or https:// means you've safely connected to the .gov website. Share sensitive information only on official, secure websites.

View full-text article in PMC

. 2015 Jul 23;24(19):5524–5541. doi: 10.1093/hmg/ddv283

Search in PMC
Search in PubMed
View in NLM Catalog
Add to search

Published by Oxford University Press 2015. This work is written by (a) US Government employee(s) and is in the public domain in the US.

PMC Copyright notice

Figure 7. — Electrophysiological measures of motor unit function following targeted Smn-deletion and restoration. (A) Compound muscle action potential (CMAP) at P21 in mice with Smn-deletion. Compound muscle action potential (CMAP) amplitudes at postnatal day 21 (P21) were reduced in ChAT-Cre Smn-deletion (n = 9, 20.4 ± 1.9, P < 0.05) and Nestin-Cre + ChAT-Cre Smn-deletion (n = 13, 26.1 ± 2.1, P = <0.05) but not Nestin-Cre Smn-deletion mice (n = 14, 35.7 ± 3.7) compared with controls (n = 13, 44.0 ± 2.9 mV). CMAP amplitudes in ChAT-Cre Smn-deletion mice were reduced compared with Nestin-Cre Smn-deletion mice (P < 0.05). (B) Motor unit number estimation (MUNE) at P21 in mice with Smn-deletion. Motor unit number estimates (MUNE) at P21 were reduced in ChAT-Cre Smn-deletion (n = 9, 142 ± 22), Nestin-Cre + ChAT-Cre Smn-deletion (n = 13, 117 ± 15), and Nestin-Cre Smn-deletion mice (n = 14, 179 ± 24) compared with controls (n = 13, 283 ± 15) (P < 0.001). (C) Electromyography of limb muscles of mice with Smn deletion. Fibrillations were noted in both ChAT-Cre Smn-deletion (four of five mice) and Nestin-Cre + ChAT-Cre Smn-deletion mice (six of seven mice) but are absent in Nestin-Cre Smn-deletion mice (zero of six) and control mice (zero of five) (P < 0.05). (D) CMAP amplitudes at P12 in mice with Smn-restoration. CMAP amplitudes at P12 in mice with Nestin-Cre + ChAT-Cre Smn-restoration (n = 9, 23.2 ± 3.3) were similar to control mice (n = 13, 24.8 ± 1.2 mV). Conversely, compared with control mice, CMAP amplitudes were reduced in mice with Nestin-Cre-restoration of Smn (n = 8, 17.3 ± 1.1 mV) and in SMA mice with no Cre (i.e. no Smn-restoration) (n = 12, 17.1 ± 0.8 mV) (P < 0.05). CMAP amplitudes were reduced though does not reach statistical significance in SMA mice with ChAT-Cre-restoration of Smn (n = 14, 18.8 ± 1.2 mV). (E) MUNE at P12 in mice with Smn-restoration. MUNE at P12 in mice with ChAT-Cre Smn-restoration (n = 14, 187 ± 16) and Nestin-Cre + ChAT-Cre Smn-restoration (n = 9, 201 ± 22) are similar to controls (n = 13, 196 ± 16, P = 0.962), but MUNE are reduced in mice with Nestin-Cre-restoration of Smn (n = 8, 118 ± 16) and SMA mice with no Cre (i.e. no Smn-restoration) (119 ± 9) (P < 0.05). Compared with mice with Nestin-Cre-restoration of Smn, MUNE at P12 in mice with ChAT-Cre Smn-restoration (P = 0.042) and Nestin-Cre + ChAT-Cre Smn-restoration (P = 0.024) are increased. Similarly, compared with SMA mice with no Cre, MUNE in mice with ChAT-Cre Smn-restoration (P = 0.031) and Nestin-Cre + ChAT-Cre Smn-restoration (P = 0.017) are increased. (*P ≤ 0.05, ***P ≤ 0.001).