HDAC6 deficiency results in accumulation of SOD1 G93A mutant protein aggregates in the spinal cord (A) 129/Black Swiss/B6SJL background mice for each genotype were sacrificed at 20 weeks of age, and dissected spinal cord were subjected to the immunostaining with anti-ubiquitin antibody for ubiquitinated protein aggregates. Arrowheads indicate ubiquitinated protein aggregates. (B) Spinal cord lysates (100 ug) from indicated genotypes at 20 weeks old were subjected to filter trap assay to measure protein aggregates, using anti-ubiquitin (bottom panel) and anti-human SOD1 antibody (top panel). (C) The same spinal cord lysates were subjected to immunoblotting using anti-human SOD1, anti-HDAC6, anti-acetyl tubulin and anti-actin antibodies.
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