Surface plot profiles of SMN and gemins 2–4 staining in the rat fALS model employed.

<p>Pseudocolors show staining intensity.</p

Immunostaining of SMN and gemins in the spinal cord of sALS patient.

<p>Immunofluorescence shows co-expression of SMN and individual gemins in spinal cord motoneurons of a 52 years-old patient who died with a 1.5 years history of sALS.</p

Changes in number of surviving motoneurons (MNs) in spinal cord ventral horns of transgenic <i>SOD1^G93A</i> male rats over the course of the model disease.

a<p>In relation to the mean MNs number in the transgenic rats 60 days of age;</p><p>**p<0.01,</p><p>***p<0.001;</p><p>one-way ANOVA (F_3,8 = 42.6, p<0.001) followed by Dunnett’s test.</p

Immunostaining for SMN and gemins in the spinal cords of symptomatic fALS rats.

<p>Immunofluorescence shows co-expression of SMN and individual gemins 2–4 in spinal cord motoneurons of >120-day-old fALS rats.</p

SMN and other gemins immunostaining intensity in spinal cord anterior horns motoneurons in sALS patients in relation to the duration of the disease.

a<p>The intensity of specific brown immunostaining was graded as follows:</p><p>+++ - very intense,</p><p>++ - intense,</p><p>+ - moderate,</p><p>+/- - low,</p><p>–/+ - vestigial,</p><p>– - absent.</p

Expression of SMN and gemins 2–4 in the rat fALS model employed.

<p>Note decreasing immunostaining for gemins with the disease duration and progress.</p

Basic clinical features of the ALS patients and controls covered by the study, and numbers of surviving motoneurons (MNs) in the anterior horns of their spinal cord cervical enlargement (C4–C8 level).

a<p>This period included the 2-hour period immediately following the death, which the bodies, as required by the Polish law, spent at room temperature before being transferred to hospital mortuary refrigerator.</p>b<p>In relation to the averaged mean number of surviving MNs in controls.</p

Surface plot profiles of SMN and gemins 2–4 staining in patients with sALS.

<p>Pseudocolors show staining intensity.</p

Expression of SMN and gemins 2–4 in patients with sALS.

<p>Note very low or absent immunostaining for gemin 2 in comparison to the other studied subunits of the SMN complex.</p