Found: 23
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Inducible transgenic expression of tripeptidyl peptidase 1 in a mouse model of late-infantile neuronal ceroid lipofuscinosis.
- Published in:
- PLoS ONE, 2018, v. 13, n. 2, p. 1, doi. 10.1371/journal.pone.0192286
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- Article
Novel Polyanionic Tracts Improve Expression of a TPP1:K<sub>16</sub>ApoE Chimera.
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- FASEB Journal, 2022, v. 36, p. N.PAG, doi. 10.1096/fasebj.2022.36.S1.00R48
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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
A mutation in the ovine cathepsin D gene causes a congenital lysosomal storage disease with profound neurodegeneration.
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- EMBO Journal, 2000, v. 19, n. 12, p. 2786, doi. 10.1093/emboj/19.12.2786
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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
Lysosomal protein thermal stability does not correlate with cellular half-life: global observations and a case study of tripeptidyl-peptidase 1.
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- Biochemical Journal, 2020, v. 477, n. 3, p. 727, doi. 10.1042/BCJ20190874
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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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- Publication type:
- Article
Intraventricular Enzyme Replacement Improves Disease Phenotypes in a Mouse Model of Late Infantile Neuronal Ceroid Lipofuscinosis.
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- Molecular Therapy, 2008, v. 16, n. 4, p. 649, doi. 10.1038/mt.2008.9
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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
893. Second Generation Gene Therapy Vector for Classical Late Infantile Neuronal Ceroid Lipofuscinosis.
- Published in:
- Molecular Therapy, 2006, v. 13, p. S344, doi. 10.1016/j.ymthe.2006.08.982
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- Article
218. Gene Therapy and Enzyme Replacement in a Mouse Model of Late Infantile Neuronal Ceroid Lipofuscinosis.
- Published in:
- Molecular Therapy, 2006, v. 13, p. S84, doi. 10.1016/j.ymthe.2006.08.243
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- Publication type:
- Article
427. AAV2- and AAV5-Mediated CNS Delivery of Human CLN2 Reduces Lysosomal Storage in a Mouse Model of Late Infantile Neuronal Ceroid Lipofuscinosis
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- 2005
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- Abstract
Potential Pitfalls and Solutions for Use of Fluorescent Fusion Proteins to Study the Lysosome.
- Published in:
- PLoS ONE, 2014, v. 9, n. 2, p. 1, doi. 10.1371/journal.pone.0088893
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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.
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- PLoS ONE, 2011, v. 6, n. 8, p. 1, doi. 10.1371/journal.pone.0023677
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- Publication type:
- 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
A mouse mutant deficient in both neuronal ceroid lipofuscinosis‐associated proteins CLN3 and TPP1.
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- Journal of Inherited Metabolic Disease, 2023, v. 46, n. 4, p. 720, doi. 10.1002/jimd.12619
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- Article
Intracranial Delivery of CLN2 Reduces Brain Pathology in a Mouse Model of Classical Late Infantile Neuronal Ceroid Lipofuscinosis.
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- Journal of Neuroscience, 2006, v. 26, n. 5, p. 1334, doi. 10.1523/JNEUROSCI.2676-05.2006
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- Article
A Mouse Model of Classical Late-Infantile Neuronal Ceroid Lipofuscinosis Based on Targeted Disruption of the CLN2 Gene Results in a Loss of Tripeptidyl-Peptidase I Activity and Progressive Neurodegeneration.
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- Journal of Neuroscience, 2004, v. 24, n. 41, p. 9117, doi. 10.1523/JNEUROSCI.2729-04.2004
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- Publication type:
- Article
Expression and analysis of CLN2 variants in CHO cells: Q100r represents a polymorphism, and G389E and R447H represent loss-of-function mutations.
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- Human Mutation, 2001, v. 18, n. 2, p. 165, doi. 10.1002/humu.1170
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- Article
Using whole-exome sequencing to investigate the genetic bases of lysosomal storage diseases of unknown etiology.
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- Human Mutation, 2017, v. 38, n. 11, p. 1491, doi. 10.1002/humu.23291
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- Article
Lysosomal proteomics and disease.
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- Proteomics - Clinical Applications, 2007, v. 1, n. 9, p. 1134, doi. 10.1002/prca.200700250
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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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- Publication type:
- Article