gemfibrozil has been researched along with Neuronal Ceroid-Lipofuscinoses in 5 studies
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.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Gemfibrozil has shown efficacy in an animal model of NCL known as CLN2 (late infantile classic juvenile) and has been shown to be safe for lowering lipids in children." | 2.55 | Safety and potential efficacy of gemfibrozil as a supportive treatment for children with late infantile neuronal ceroid lipofuscinosis and other lipid storage disorders. ( Kim, K; Kleinman, HK; Lee, HJ; Pahan, K, 2017) |
| "Late Infantile Neuronal Ceroid Lipofuscinosis (LINCL) is a rare neurodegenerative disease caused by mutations in the Cln2 gene that leads to deficiency or loss of function of the tripeptidyl peptidase 1 (TPP1) enzyme." | 1.46 | Gemfibrozil, food and drug administration-approved lipid-lowering drug, increases longevity in mouse model of late infantile neuronal ceroid lipofuscinosis. ( Ghosh, A; Modi, KK; Pahan, K; Rangasamy, SB, 2017) |
| "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." | 1.43 | Fibrates 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)." | 1.38 | 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. ( Corbett, GT; Ghosh, A; Gonzalez, FJ; Pahan, K, 2012) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 5 (100.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Kim, K | 1 |
| Kleinman, HK | 1 |
| Lee, HJ | 1 |
| Pahan, K | 3 |
| Lojewski, X | 1 |
| Staropoli, JF | 1 |
| Biswas-Legrand, S | 1 |
| Simas, AM | 1 |
| Haliw, L | 1 |
| Selig, MK | 1 |
| Coppel, SH | 1 |
| Goss, KA | 1 |
| Petcherski, A | 1 |
| Chandrachud, U | 1 |
| Sheridan, SD | 1 |
| Lucente, D | 1 |
| Sims, KB | 1 |
| Gusella, JF | 1 |
| Sondhi, D | 1 |
| Crystal, RG | 1 |
| Reinhardt, P | 1 |
| Sterneckert, J | 1 |
| Schöler, H | 1 |
| Haggarty, SJ | 1 |
| Storch, A | 1 |
| Hermann, A | 1 |
| Cotman, SL | 1 |
| Hong, M | 1 |
| Song, KD | 1 |
| Lee, HK | 1 |
| Yi, S | 1 |
| Lee, YS | 1 |
| Heo, TH | 1 |
| Jun, HS | 1 |
| Kim, SJ | 1 |
| Ghosh, A | 2 |
| Rangasamy, SB | 1 |
| Modi, KK | 1 |
| Corbett, GT | 1 |
| Gonzalez, FJ | 1 |
1 review available for gemfibrozil and Neuronal Ceroid-Lipofuscinoses
| Article | Year |
|---|---|
Safety and potential efficacy of gemfibrozil as a supportive treatment for children with late infantile neuronal ceroid lipofuscinosis and other lipid storage disorders.
Topics: Child; Gemfibrozil; Humans; Neuronal Ceroid-Lipofuscinoses; Tripeptidyl-Peptidase 1 | 2017 |
4 other studies available for gemfibrozil and Neuronal Ceroid-Lipofuscinoses
| Article | Year |
|---|---|
Human iPSC models of neuronal ceroid lipofuscinosis capture distinct effects of TPP1 and CLN3 mutations on the endocytic pathway.
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.
Topics: Apoptosis; Autophagy; Bezafibrate; Cells, Cultured; Fenofibrate; Gemfibrozil; Humans; Lymphocytes; M | 2016 |
Gemfibrozil, food and drug administration-approved lipid-lowering drug, increases longevity in mouse model of late infantile neuronal ceroid lipofuscinosis.
Topics: Aminopeptidases; Animals; Apoptosis; bcl-Associated Death Protein; Dipeptidyl-Peptidases and Tripept | 2017 |
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.
Topics: Aminopeptidases; Animals; Astrocytes; Brain; Dipeptidyl-Peptidases and Tripeptidyl-Peptidases; Fenof | 2012 |