GLB1 and mucopolysaccharidosis: Notably, the activities of 2 other LMC components, GLB1 and GALNS, stabilized by their association with CTSA (23), were reduced by at least 50% in brain tissues of patients with MPS and cultured iPSC-derived MPS IIIA neurons; in HgsnatP304L mice brain extracts, however, GLB1 was present in the form of homotetramers instead of LMC.