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. Author manuscript; available in PMC: 2018 Aug 7.
Published in final edited form as: Dev Cell. 2017 Aug 7;42(3):286–300.e4. doi: 10.1016/j.devcel.2017.07.010

Figure 7. The effect of Jourbert-Sydrome linked human ARL13B alleles in Arl13b deficient interneurons.

Figure 7

(A) Schematic of human ARL13B mutations and mouse non-cilia targeted Arl13b. (B–I) Dissociated INs from Nkx2.1Cre; Arl13blox/+; Ai9 (B) or Nkx2.1Cre; Arl13blox/lox; Ai9 (C–I) mice were transfected with AAV-mCherry (B, C), AAV-mCherry-ARL13B (D), R79Q (E), W82X (F), Y86C (G), R200C (H), and mV358A (I). Inset (I) shows non-ciliary expression of Arl13bV358A. (B′–I′) Labeling with anti-VGAT antibodies show inhibitory presynaptic boutons (arrowhead) along mCherry+ IN axons (B–I). (J, K) Quantification of total dendritic (J) and axonal length (K). (L, M) Quantification of VGAT+ bouton density (L) and size (M) along axons. Data shown are mean ± SEM. 12 cells from 3 independent experiments were analyzed per group. *P<0.05 (One-way ANOVA: F7,88 = 13.27[J], 17.61[K], 25.75[L], 23.52[M]; p = 1.43E-11[J], 2.11E-14[K], 8.13E-19[L], 1.06E-17[M]; post-hoc p[lox/+ vs. lox/lox = 0.001[J], 7.50E-08[K], 2.34E-06[L], 0.0003[M]; post-hoc p[lox/+ vs. lox/lox-ARL13B = 0.06[J], 0.1[K], 0.08[L], 0.09[M]; post-hoc p[lox/+ vs. lox/lox-R79Q] = 9.38E-06 [J], 6.50E-05[K], 6.44E-06[L], 0.0003[M]; post-hoc p[lox/+ vs. lox/lox-W82X] = 0.009 [J], 3.51E-08[K], 1.15E-05[L], 2.14E-06[M]; post-hoc p[lox/+ vs. lox/lox-Y86C] = 0.002 [J], 6.42E-06[K], 7.67E-08[L], 9.10E-06[M]; post-hoc p[lox/+ vs. lox/lox-R200C] = 0.46[J], 0.06[K], 0.002[L], 0.004[M]; post-hoc p[lox/+ vs. lox/lox-V358A] = 0.0001[J], 6.91E-05[K], 5.2771E-08[L], 1.63E-05[M]. Scale bar, 40μm (B–I); 10μm (B′–I′).