Shotgun proteomics reveals possible mechanisms for cognitive impairment in Mucopolysaccharidosis I mice
G Baldo, DM Lorenzini, DS Santos, FQ Mayer… - Molecular Genetics and …, 2015 - Elsevier
Abstract Mucopolysaccharidosis type I (MPS I) is due to deficient alpha-L-iduronidase
(IDUA) which leads to storage of undegraded glycosaminoglycans (GAG). The severe form
of the disease is characterized by mental retardation of unknown etiology. Trying to unveil
the mechanisms that lead to cognitive impairment in MPS I, we studied alterations in the
proteome from MPS I mouse hippocampus. Eight-month old mice presented increased
LAMP-1 expression, GAG storage in neurons and glial cells, and impaired aversive and non …
(IDUA) which leads to storage of undegraded glycosaminoglycans (GAG). The severe form
of the disease is characterized by mental retardation of unknown etiology. Trying to unveil
the mechanisms that lead to cognitive impairment in MPS I, we studied alterations in the
proteome from MPS I mouse hippocampus. Eight-month old mice presented increased
LAMP-1 expression, GAG storage in neurons and glial cells, and impaired aversive and non …