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fenofibrate and Neuronal Ceroid-Lipofuscinoses

fenofibrate has been researched along with Neuronal Ceroid-Lipofuscinoses in 3 studies

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Neuronal Ceroid-Lipofuscinoses: A group of severe neurodegenerative diseases characterized by intracellular accumulation of autofluorescent wax-like lipid materials (CEROID; LIPOFUSCIN) in neurons. There are several subtypes based on mutations of the various genes, time of disease onset, and severity of the neurological defects such as progressive DEMENTIA; SEIZURES; and visual failure.

Research Excerpts

ExcerptRelevanceReference
"Batten disease (BD; also known as juvenile neuronal ceroid lipofuscinosis) is a genetic disorder inherited as an autosomal recessive trait and is characterized by blindness, seizures, cognitive decline, and early death resulting from the inherited mutation of the CLN3 gene."1.43Fibrates inhibit the apoptosis of Batten disease lymphoblast cells via autophagy recovery and regulation of mitochondrial membrane potential. ( Heo, TH; Hong, M; Jun, HS; Kim, SJ; Lee, HK; Lee, YS; Song, KD; Yi, S, 2016)
"The classical late infantile neuronal ceroid lipofuscinosis (LINCLs) is an autosomal recessive disease, where the defective gene is Cln2, encoding tripeptidyl-peptidase I (TPP1)."1.38Gemfibrozil 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. ( Corbett, GT; Ghosh, A; Gonzalez, FJ; Pahan, K, 2012)

Research

Studies (3)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's3 (100.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Lojewski, X1
Staropoli, JF1
Biswas-Legrand, S1
Simas, AM1
Haliw, L1
Selig, MK1
Coppel, SH1
Goss, KA1
Petcherski, A1
Chandrachud, U1
Sheridan, SD1
Lucente, D1
Sims, KB1
Gusella, JF1
Sondhi, D1
Crystal, RG1
Reinhardt, P1
Sterneckert, J1
Schöler, H1
Haggarty, SJ1
Storch, A1
Hermann, A1
Cotman, SL1
Hong, M1
Song, KD1
Lee, HK1
Yi, S1
Lee, YS1
Heo, TH1
Jun, HS1
Kim, SJ1
Ghosh, A1
Corbett, GT1
Gonzalez, FJ1
Pahan, K1

Other Studies

3 other studies available for fenofibrate and Neuronal Ceroid-Lipofuscinoses

ArticleYear
Human iPSC models of neuronal ceroid lipofuscinosis capture distinct effects of TPP1 and CLN3 mutations on the endocytic pathway.
    Human molecular genetics, 2014, Apr-15, Volume: 23, Issue:8

    Topics: Aminopeptidases; Blotting, Western; Case-Control Studies; Cell Proliferation; Cells, Cultured; Dipep

2014
Fibrates inhibit the apoptosis of Batten disease lymphoblast cells via autophagy recovery and regulation of mitochondrial membrane potential.
    In vitro cellular & developmental biology. Animal, 2016, Volume: 52, Issue:3

    Topics: Apoptosis; Autophagy; Bezafibrate; Cells, Cultured; Fenofibrate; Gemfibrozil; Humans; Lymphocytes; M

2016
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 Journal of biological chemistry, 2012, Nov-09, Volume: 287, Issue:46

    Topics: Aminopeptidases; Animals; Astrocytes; Brain; Dipeptidyl-Peptidases and Tripeptidyl-Peptidases; Fenof

2012