Mucopolysaccharidosis type I: Current knowledge on its pathophysiological mechanisms

Metabolic Brain Disease

Volumn 27, Issue 2, 2012, Pages 121-129

  Campos, Derbis ^a   Monaga, Madelyn ^a  

^a Campus ICBP   (Cuba)

Author keywords

Lysosomal storage diseases; MPS I; Neuro synaptic dysfunction; Neurodegeneration; Pathophysiology

Indexed keywords

CALCIUM ION; GANGLIOSIDE GM1; GANGLIOSIDE GM2; GLYCOSAMINOGLYCAN;

APOPTOSIS; BRAIN ATROPHY; BRAIN VENTRICLE DILATATION; CALCIUM TRANSPORT; CARPAL TUNNEL SYNDROME; CELL MEMBRANE PERMEABILITY; CYTOLOGY; HISTOPATHOLOGY; HUMAN; HURLER SYNDROME; HYDROCEPHALUS; HYPOTHALAMUS PERIVENTRICULAR NUCLEUS; INFLAMMATION; INTELLECTUAL IMPAIRMENT; INTRACELLULAR SIGNALING; INTRACRANIAL HYPERTENSION; LIPID DEGRADATION; LIPID STORAGE; LYSOSOME; LYSOSOME STORAGE DISEASE; MACROPHAGE; MICROGLIA; NONHUMAN; NUCLEAR MAGNETIC RESONANCE IMAGING; OPTIC NERVE ATROPHY; OXIDATIVE STRESS; PATHOPHYSIOLOGY; REVIEW; SPINAL CORD COMPRESSION; SPIRAL GANGLION; VESTIBULOCOCHLEAR NERVE;

ANIMALS; APOPTOSIS; GANGLIOSIDES; GLYCOSAMINOGLYCANS; HOMEOSTASIS; HUMANS; INFLAMMATION; LYSOSOME-ASSOCIATED MEMBRANE GLYCOPROTEINS; LYSOSOMES; MUCOPOLYSACCHARIDOSIS I; SIGNAL TRANSDUCTION;

ANIMALIA;

EID: 84863459850     PISSN: 08857490     EISSN: 15737365     Source Type: Journal    
DOI: 10.1007/s11011-012-9302-1     Document Type: Review

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