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A mutation in the ovine cathepsin D gene causes a congenital lysosomal storage disease with profound neurodegeneration.
- Published in:
- EMBO Journal, 2000, v. 19, n. 12, p. 2786, doi. 10.1093/emboj/19.12.2786
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- Article
Systemic Administration of Tripeptidyl Peptidase I in a Mouse Model of Late Infantile Neuronal Ceroid Lipofuscinosis: Effect of Glycan Modification.
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- PLoS ONE, 2012, v. 7, n. 7, p. 1, doi. 10.1371/journal.pone.0040509
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- Article
Loss of Niemann-Pick C1 or C2 Protein Results in Similar Biochemical Changes Suggesting That These Proteins Function in a Common Lysosomal Pathway.
- Published in:
- PLoS ONE, 2011, v. 6, n. 8, p. 1, doi. 10.1371/journal.pone.0023677
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- Article
Proteomic analysis of mouse models of Niemann- Pick C disease reveals alterations in the steady-state levels of lysosomal proteins within the brain.
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- Proteomics, 2012, v. 12, n. 23/24, p. 3499, doi. 10.1002/pmic.201200205
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- Article
Effective Intravenous Therapy for Neurodegenerative Disease With a Therapeutic Enzyme and a Peptide That Mediates Delivery to the Brain.
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- Molecular Therapy, 2014, v. 22, n. 3, p. 547, doi. 10.1038/mt.2013.267
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- Article
Large-volume Intrathecal Enzyme Delivery Increases Survival of a Mouse Model of Late Infantile Neuronal Ceroid Lipofuscinosis.
- Published in:
- Molecular Therapy, 2011, v. 19, n. 10, p. 1842, doi. 10.1038/mt.2011.130
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- Article
Timing of Therapeutic Intervention Determines Functional and Survival Outcomes in a Mouse Model of Late Infantile Batten Disease.
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- Molecular Therapy, 2007, v. 15, n. 10, p. 1782, doi. 10.1038/sj.mt.6300249
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- Article
Prenatal testing for late infantile neuronal ceroid lipofuscinosis.
- Published in:
- Annals of Neurology, 2000, v. 47, n. 2, p. 254, doi. 10.1002/1531-8249(200002)47:2<254::AID-ANA19>3.0.CO;2-7
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- Article
Biochemical Characterization of a Lysosomal Protease Deficient in Classical Late Infantile Neuronal Ceroid Lipofuscinosis (LINCL) and Development of an Enzyme-Based Assay for Diagnosis and Exclusion of LINCL in HUman Specimens and Animal Models.
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- Journal of Neurochemistry, 1999, v. 73, n. 2, p. 700, doi. 10.1046/j.1471-4159.1999.0730700.x
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- Article
REGULATION OF PROTEINASE ACTIVATION IN MAMMALIAN TISSUES.
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- Biological Chemistry, 1992, v. 373, n. 2, p. 567
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- Article