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1.
A mouse mutant deficient in both neuronal ceroid lipofuscinosis-associated proteins CLN3 and TPP1.
J Inherit Metab Dis
; 46(4): 720-734, 2023 07.
Artículo
en Inglés
| MEDLINE | ID: mdl-37078466
2.
Analysis of Brain and Cerebrospinal Fluid from Mouse Models of the Three Major Forms of Neuronal Ceroid Lipofuscinosis Reveals Changes in the Lysosomal Proteome.
Mol Cell Proteomics
; 18(11): 2244-2261, 2019 11.
Artículo
en Inglés
| MEDLINE | ID: mdl-31501224
3.
A Basic ApoE-Based Peptide Mediator to Deliver Proteins across the Blood-Brain Barrier: Long-Term Efficacy, Toxicity, and Mechanism.
Mol Ther
; 25(7): 1531-1543, 2017 07 05.
Artículo
en Inglés
| MEDLINE | ID: mdl-28456380
4.
Proteomic Analysis of Brain and Cerebrospinal Fluid from the Three Major Forms of Neuronal Ceroid Lipofuscinosis Reveals Potential Biomarkers.
J Proteome Res
; 16(10): 3787-3804, 2017 10 06.
Artículo
en Inglés
| MEDLINE | ID: mdl-28792770
5.
Extending the mannose 6-phosphate glycoproteome by high resolution/accuracy mass spectrometry analysis of control and acid phosphatase 5-deficient mice.
Mol Cell Proteomics
; 12(7): 1806-17, 2013 Jul.
Artículo
en Inglés
| MEDLINE | ID: mdl-23478313
6.
Proteomic analysis of mouse models of Niemann-Pick C disease reveals alterations in the steady-state levels of lysosomal proteins within the brain.
Proteomics
; 12(23-24): 3499-509, 2012 Dec.
Artículo
en Inglés
| MEDLINE | ID: mdl-23070805
7.
Inducible transgenic expression of tripeptidyl peptidase 1 in a mouse model of late-infantile neuronal ceroid lipofuscinosis.
PLoS One
; 13(2): e0192286, 2018.
Artículo
en Inglés
| MEDLINE | ID: mdl-29408933
8.
Chronic Enzyme Replacement to the Brain of a Late Infantile Neuronal Ceroid Lipofuscinosis Mouse Has Differential Effects on Phenotypes of Disease.
Mol Ther Methods Clin Dev
; 4: 204-212, 2017 Mar 17.
Artículo
en Inglés
| MEDLINE | ID: mdl-28345005
9.
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.
J Neurosci
; 24(41): 9117-26, 2004 Oct 13.
Artículo
en Inglés
| MEDLINE | ID: mdl-15483130
10.
Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport.
Proc Natl Acad Sci U S A
; 101(16): 5886-91, 2004 Apr 20.
Artículo
en Inglés
| MEDLINE | ID: mdl-15071184
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