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Grabowski, Gregory
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Item TypeName
Concept Saposins
Academic Article Saposin C is required for normal resistance of acid beta-glucosidase to proteolytic degradation.
Academic Article Gaucher disease mouse models: point mutations at the acid beta-glucosidase locus combined with low-level prosaposin expression lead to disease variants.
Academic Article Saposin C: neuronal effect and CNS delivery by liposomes.
Academic Article Combined saposin C and D deficiencies in mice lead to a neuronopathic phenotype, glucosylceramide and alpha-hydroxy ceramide accumulation, and altered prosaposin trafficking.
Academic Article Neurological deficits and glycosphingolipid accumulation in saposin B deficient mice.
Academic Article Temporal gene expression profiling reveals CEBPD as a candidate regulator of brain disease in prosaposin deficient mice.
Academic Article Specific saposin C deficiency: CNS impairment and acid beta-glucosidase effects in the mouse.
Academic Article Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficits.
Academic Article The role of UDP-Glc:glycoprotein glucosyltransferase 1 in the maturation of an obligate substrate prosaposin.
Academic Article Comparative therapeutic effects of velaglucerase alfa and imiglucerase in a Gaucher disease mouse model.
Academic Article Saposins utilize two strategies for lipid transfer and CD1 antigen presentation.
Academic Article Tissue-specific effects of saposin A and saposin B on glycosphingolipid degradation in mutant mice.
Academic Article Altered autophagy in the mice with a deficiency of saposin A and saposin B.
Academic Article Spiral ganglion degeneration and hearing loss as a consequence of satellite cell death in saposin B-deficient mice.
Academic Article Role of saposin C and D in auditory and vestibular function.
Academic Article Combination of acid ?-glucosidase mutation and Saposin C deficiency in mice reveals Gba1 mutation dependent and tissue-specific disease phenotype.
Academic Article Ex vivo localization of the mouse saposin C activation region for acid beta-glucosidase.
Academic Article Analyses of temporal regulatory elements of the prosaposin gene in transgenic mice.
Academic Article Systemic enzyme delivery by blood-brain barrier-penetrating SapC-DOPS nanovesicles for treatment of neuronopathic Gaucher disease.
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