Inducible transgenic expression of tripeptidyl peptidase 1 in a mouse model of late-infantile neuronal ceroid lipofuscinosis

PLoS ONE ◽

10.1371/journal.pone.0192286 ◽

2018 ◽

Vol 13 (2) ◽

pp. e0192286 ◽

Author(s):

Yuliya Nemtsova ◽

Jennifer A. Wiseman ◽

Mukarram El-Banna ◽

Peter Lobel ◽

David E. Sleat

Keyword(s):

Mouse Model ◽

Neuronal Ceroid Lipofuscinosis ◽

Transgenic Expression ◽

Infantile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis ◽

Tripeptidyl Peptidase

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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

Journal of Neuroscience ◽

10.1523/jneurosci.2729-04.2004 ◽

2004 ◽

Vol 24 (41) ◽

pp. 9117-9126 ◽

Author(s):

D. E. Sleat

Keyword(s):

Mouse Model ◽

Neuronal Ceroid Lipofuscinosis ◽

Targeted Disruption ◽

Infantile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis ◽

Tripeptidyl Peptidase ◽

Progressive Neurodegeneration

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Systemic Administration of Tripeptidyl Peptidase I in a Mouse Model of Late Infantile Neuronal Ceroid Lipofuscinosis: Effect of Glycan Modification

PLoS ONE ◽

10.1371/journal.pone.0040509 ◽

2012 ◽

Vol 7 (7) ◽

pp. e40509 ◽

Author(s):

Yu Meng ◽

Istvan Sohar ◽

Lingling Wang ◽

David E. Sleat ◽

Peter Lobel

Keyword(s):

Mouse Model ◽

Neuronal Ceroid Lipofuscinosis ◽

Systemic Administration ◽

Infantile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis ◽

Tripeptidyl Peptidase ◽

Glycan Modification

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218. Gene Therapy and Enzyme Replacement in a Mouse Model of Late Infantile Neuronal Ceroid Lipofuscinosis

Molecular Therapy ◽

10.1016/j.ymthe.2006.08.243 ◽

2006 ◽

Vol 13 ◽

pp. S84

Author(s):

Michael Chang ◽

David Sleat ◽

Seng Cheng ◽

Marco Passini ◽

Peter Lobel ◽

...

Keyword(s):

Gene Therapy ◽

Mouse Model ◽

Neuronal Ceroid Lipofuscinosis ◽

Infantile Neuronal Ceroid Lipofuscinosis ◽

Enzyme Replacement ◽

Ceroid Lipofuscinosis

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First-trimester diagnosis of late-infantile neuronal ceroid lipofuscinosis (LINCL) by tripeptidyl peptidase I assay and CLN2 mutation analysis

Prenatal Diagnosis ◽

10.1002/1097-0223(200102)21:2<99::aid-pd988>3.0.co;2-f ◽

2001 ◽

Vol 21 (2) ◽

pp. 99-101 ◽

Author(s):

W. J. Kleijer ◽

O. P. van Diggelen ◽

J. L. M. Keulemans ◽

M. Losekoot ◽

V. H. Garritsen ◽

...

Keyword(s):

Mutation Analysis ◽

Neuronal Ceroid Lipofuscinosis ◽

First Trimester ◽

Infantile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis ◽

Tripeptidyl Peptidase

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Profound loss of GABAergic interneurons in the PPT1 knockout mouse model of infantile neuronal ceroid lipofuscinosis

Neuropathology and Applied Neurobiology ◽

10.1046/j.1365-2990.2002.39286_29.x ◽

2002 ◽

Vol 28 (2) ◽

pp. 158-159 ◽

Author(s):

J. D. Cooper ◽

P. Gupta ◽

E. Bible ◽

S. Hofmann ◽

P. Lantos) ◽

...

Keyword(s):

Mouse Model ◽

Knockout Mouse ◽

Neuronal Ceroid Lipofuscinosis ◽

Gabaergic Interneurons ◽

Knockout Mouse Model ◽

Infantile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis

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The blood-brain barrier is disrupted in a mouse model of infantile neuronal ceroid lipofuscinosis: amelioration by resveratrol

Human Molecular Genetics ◽

10.1093/hmg/dds038 ◽

2012 ◽

Vol 21 (10) ◽

pp. 2233-2244 ◽

Author(s):

Arjun Saha ◽

Chinmoy Sarkar ◽

Satya P. Singh ◽

Zhongjian Zhang ◽

Jeeva Munasinghe ◽

...

Keyword(s):

Mouse Model ◽

Blood Brain Barrier ◽

Neuronal Ceroid Lipofuscinosis ◽

Brain Barrier ◽

Infantile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis ◽

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Intrathecal and intravenous combination gene therapy in the mouse model of infantile neuronal ceroid lipofuscinosis extends lifespan and improves behavioral outcomes in moderately affected mice

Molecular Genetics and Metabolism ◽

10.1016/j.ymgme.2018.12.149 ◽

2019 ◽

Vol 126 (2) ◽

pp. S63-S64

Author(s):

Steven J. Gray ◽

Alejandra Rozenberg ◽

Erik Lykken ◽

Linas Padegimas ◽

Timothy Miller

Keyword(s):

Gene Therapy ◽

Mouse Model ◽

Neuronal Ceroid Lipofuscinosis ◽

Behavioral Outcomes ◽

Infantile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis

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Gene expression profiling in a mouse model of infantile neuronal ceroid lipofuscinosis reveals upregulation of immediate early genes and mediators of the inflammatory response

BMC Neuroscience ◽

10.1186/1471-2202-8-95 ◽

2007 ◽

Vol 8 (1) ◽

pp. 95 ◽

Author(s):

Xingwen Qiao ◽

Jui-Yun Lu ◽

Sandra L Hofmann

Keyword(s):

Gene Expression ◽

Inflammatory Response ◽

Mouse Model ◽

Gene Expression Profiling ◽

Expression Profiling ◽

Neuronal Ceroid Lipofuscinosis ◽

Immediate Early Genes ◽

Immediate Early ◽

Infantile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis

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427. AAV2- and AAV5-Mediated CNS Delivery of Human CLN2 Reduces Lysosomal Storage in a Mouse Model of Late Infantile Neuronal Ceroid Lipofuscinosis

Molecular Therapy ◽

10.1016/j.ymthe.2005.06.429 ◽

2005 ◽

Vol 11 ◽

pp. S166

Keyword(s):

Mouse Model ◽

Neuronal Ceroid Lipofuscinosis ◽

Lysosomal Storage ◽

Infantile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis ◽

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The lysosomal degradation of neuromedin B is dependent on tripeptidyl peptidase-I: evidence for the impairment of neuropeptide degradation in late-infantile neuronal ceroid lipofuscinosis

Biochemical and Biophysical Research Communications ◽

10.1016/j.bbrc.2004.04.142 ◽

2004 ◽

Vol 319 (1) ◽

pp. 58-65 ◽

Author(s):

Sharmila Kopan ◽

Uthayatharsini Sivasubramaniam ◽

Michael J Warburton

Keyword(s):

Neuronal Ceroid Lipofuscinosis ◽

Lysosomal Degradation ◽

Infantile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis ◽

Tripeptidyl Peptidase ◽

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