Figure 1.
Behavioral abnormalities and brain histopathology in an Arl6ip1−/−mouse model of HSP. (A) ARL6IP1 protein structure. Locations of mutations previously identified in patients with HSP. (B) Representative body and brain size of Arl6ip1–/– and Arl6ip1+/+ mice at 9 mo. CTX, cerebral cortex; CB, cerebellum. (C and D) Measurement of body (C) and brain/body weight ratio (D) change over time of Arl6ip1−/− and Arl6ip1+/+ mice. Data are presented as mean ± SD of n = 10 mice/group. (E) Representative image of abnormal limb reflexes in 9-mo-old Arl6ip1−/− and Arl6ip1+/+ mice. (F) Abnormal hindlimb posture of Arl6ip1−/− and Arl6ip1+/+ mice. (G) Representative image (top) and measurement of foot-base angle (bottom) of Arl6ip1−/− and Arl6ip1+/+ mice. Data are presented as mean ± SD of n = 10 mice/group. (H) Representative image of disturbed footprint pattern in Arl6ip1−/− and Arl6ip1+/+ mice (left). Forelimbs are marked in red and hindlimbs in blue. Stride, stance, and sway lengths were calculated from footprint patterns of Arl6ip1−/− and Arl6ip1+/+ mice (right). Data represent mean ± SD of n = 10 mice/group. (I) Mouse coronal section image from Allen Adult Mouse Brain Atlas (http://atlas.brain-map.org) at the same slice position as primary motor cortex and hippocampus regions (left, marked in purple). Representative images of immunofluorescence staining of NeuN and GFAP in the primary motor cortex (top) and hippocampus (down) regions of Arl6ip1−/− and Arl6ip1+/+ mice (200×, scale bar: 100 μm). (J) Representative immunofluorescence image showing MAP2 expression in primary cortical neurons from Arl6ip1−/− and Arl6ip1+/+ mice (400×, scale bar: 50 μm). (K) Neurite length and neuron cell number in WT and Arl6ip1-deficient cortical neurons. Data represent mean ± SD of triplicates and are analyzed using Student’s t test. *P < 0.05; **P < 0.01; ns, not significant. Data represent averages of three independent biological replicates with two technical replicates each.

Behavioral abnormalities and brain histopathology in an Arl6ip1 −/− mouse model of HSP. (A) ARL6IP1 protein structure. Locations of mutations previously identified in patients with HSP. (B) Representative body and brain size of Arl6ip1–/– and Arl6ip1+/+ mice at 9 mo. CTX, cerebral cortex; CB, cerebellum. (C and D) Measurement of body (C) and brain/body weight ratio (D) change over time of Arl6ip1−/− and Arl6ip1+/+ mice. Data are presented as mean ± SD of n = 10 mice/group. (E) Representative image of abnormal limb reflexes in 9-mo-old Arl6ip1−/− and Arl6ip1+/+ mice. (F) Abnormal hindlimb posture of Arl6ip1−/− and Arl6ip1+/+ mice. (G) Representative image (top) and measurement of foot-base angle (bottom) of Arl6ip1−/− and Arl6ip1+/+ mice. Data are presented as mean ± SD of n = 10 mice/group. (H) Representative image of disturbed footprint pattern in Arl6ip1−/− and Arl6ip1+/+ mice (left). Forelimbs are marked in red and hindlimbs in blue. Stride, stance, and sway lengths were calculated from footprint patterns of Arl6ip1−/− and Arl6ip1+/+ mice (right). Data represent mean ± SD of n = 10 mice/group. (I) Mouse coronal section image from Allen Adult Mouse Brain Atlas (http://atlas.brain-map.org) at the same slice position as primary motor cortex and hippocampus regions (left, marked in purple). Representative images of immunofluorescence staining of NeuN and GFAP in the primary motor cortex (top) and hippocampus (down) regions of Arl6ip1−/− and Arl6ip1+/+ mice (200×, scale bar: 100 μm). (J) Representative immunofluorescence image showing MAP2 expression in primary cortical neurons from Arl6ip1−/− and Arl6ip1+/+ mice (400×, scale bar: 50 μm). (K) Neurite length and neuron cell number in WT and Arl6ip1-deficient cortical neurons. Data represent mean ± SD of triplicates and are analyzed using Student’s t test. *P < 0.05; **P < 0.01; ns, not significant. Data represent averages of three independent biological replicates with two technical replicates each.

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