SS18L1 and amyotrophic lateral sclerosis: Importantly, we demonstrate that microglial appearance exhibits activated morphology including the enlargement of cell bodies and the decreased complexity of processes in both CREST knockout (KO) mice and homozygous CrestQ394X/Q394X (Abbreviated as Q394X) mice, suggesting that CREST haploinsufficiency or ALS-related mutation leads to microglial activation and sustains the proinflammatory state in CNS.