Individual groups have reported substantially elevated chitotriosidase (CHIT1), chitinase-3-like-1 (CHI3L1), and chitinase-3-like-2 (CHI3L2) levels in the cerebrospinal, motor cortex, and spinal cord of ALS patients with multiple—and often conflicting—lines of evidence hinting at possible links to disease severity and progression. Here, CHI3L2 is linked to amyotrophic lateral sclerosis.