Gemfibrozil and Fenofibrate, Food and Drug Administration-approved Lipid-lowering Drugs, Up-regulate Tripeptidyl-peptidase 1 in Brain Cells via Peroxisome Proliferator-activated Receptor α: IMPLICATIONS FOR LATE INFANTILE BATTEN DISEASE THERAPY

The classical late infantile neuronal ceroid lipofuscinosis (LINCLs) is an autosomal recessive disease, where the defective gene is Cln2, encoding tripeptidyl-peptidase I (TPP1). At the molecular level, LINCL is caused by accumulation of autofluorescent storage materials in neurons and other cell ty...

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Autores principales: Ghosh, Arunava, Corbett, Grant T., Gonzalez, Frank J., Pahan, Kalipada
Formato: Artigo
Lenguaje:Inglês
Publicado: American Society for Biochemistry and Molecular Biology 2012
Materias:
Enzymology
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3493933/
https://ncbi.nlm.nih.gov/pubmed/22989886
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M112.365148
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