 Neuronal Ceroid-Lipofuscinoses
"Neuronal Ceroid-Lipofuscinoses" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
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.
| Descriptor ID |
D009472
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| MeSH Number(s) |
C10.574.500.550 C16.320.400.600 C16.320.565.398.641.509 C18.452.584.687.509 C18.452.648.398.641.509
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| Concept/Terms |
Neuronal Ceroid-Lipofuscinoses- Neuronal Ceroid-Lipofuscinoses
- Neuronal Ceroid Lipofuscinoses
- Ceroid Storage Disease
- Ceroid Storage Diseases
- Disease, Ceroid Storage
- Diseases, Ceroid Storage
- Storage Disease, Ceroid
- Storage Diseases, Ceroid
- Lipofuscinosis, Neuronal Ceroid
- Neuronal Ceroid Lipofuscinosis
- Ceroid-Lipofuscinosis, Neuronal
- Ceroid Lipofuscinosis, Neuronal
- Neuronal Ceroid-Lipofuscinosis
- Lipofuscin Storage Disease
- Disease, Lipofuscin Storage
- Diseases, Lipofuscin Storage
- Lipofuscin Storage Diseases
- Storage Disease, Lipofuscin
- Storage Diseases, Lipofuscin
Late-Infantile Neuronal Ceroid Lipofuscinosis- Late-Infantile Neuronal Ceroid Lipofuscinosis
- Late Infantile Neuronal Ceroid Lipofuscinosis
- Neuronal Ceroid Lipofuscinosis, Late-Infantile
- Neuronal Ceroid Lipofuscinosis, Late Infantile
- Jansky-Bielschowsky Disease
- Jansky Bielschowsky Disease
Juvenile Neuronal Ceroid Lipofuscinosis- Juvenile Neuronal Ceroid Lipofuscinosis
- Batten-Mayou Disease
- Batten Mayou Disease
- Spielmeyer-Vogt Disease
- Spielmeyer Vogt Disease
- Juvenile Cerebroretinal Degeneration
- Cerebroretinal Degeneration, Juvenile
- Cerebroretinal Degenerations, Juvenile
- Juvenile Cerebroretinal Degenerations
- Neuronal Ceroid Lipofuscinosis, Juvenile
- Batten Disease
- Batten-Spielmeyer-Vogt Disease
- Batten Spielmeyer Vogt Disease
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Below are MeSH descriptors whose meaning is more general than "Neuronal Ceroid-Lipofuscinoses".
Below are MeSH descriptors whose meaning is more specific than "Neuronal Ceroid-Lipofuscinoses".
This graph shows the total number of publications written about "Neuronal Ceroid-Lipofuscinoses" by people in this website by year, and whether "Neuronal Ceroid-Lipofuscinoses" was a major or minor topic of these publications.
To see the data from this visualization as text, click here.
| Year | Major Topic | Minor Topic | Total |
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| 1988 | 1 | 0 | 1 | | 1989 | 1 | 0 | 1 | | 1992 | 1 | 0 | 1 | | 1993 | 1 | 0 | 1 | | 1996 | 1 | 0 | 1 | | 1997 | 1 | 0 | 1 | | 1998 | 1 | 0 | 1 | | 1999 | 1 | 0 | 1 | | 2000 | 2 | 1 | 3 | | 2001 | 1 | 0 | 1 | | 2006 | 1 | 0 | 1 | | 2007 | 1 | 0 | 1 | | 2008 | 0 | 1 | 1 | | 2009 | 1 | 0 | 1 | | 2010 | 1 | 0 | 1 | | 2015 | 1 | 0 | 1 | | 2016 | 1 | 0 | 1 | | 2019 | 1 | 0 | 1 |
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Below are the most recent publications written about "Neuronal Ceroid-Lipofuscinoses" by people in Profiles.
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Domowicz MS, Chan WC, Claudio-Vázquez P, Henry JG, Ware CB, Andrade J, Dawson G, Schwartz NB. Global Brain Transcriptome Analysis of a Tpp1 Neuronal Ceroid Lipofuscinoses Mouse Model. ASN Neuro. 2019 Jan-Dec; 11:1759091419843393.
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Metelitsina TI, Waggoner DJ, Grassi MA. BATTEN DISEASE CAUSED BY A NOVEL MUTATION IN THE PPT1 GENE. Retin Cases Brief Rep. 2016; 10(3):211-3.
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Henderson MX, Wirak GS, Zhang YQ, Dai F, Ginsberg SD, Dolzhanskaya N, Staropoli JF, Nijssen PC, Lam TT, Roth AF, Davis NG, Dawson G, Velinov M, Chandra SS. Neuronal ceroid lipofuscinosis with DNAJC5/CSPa mutation has PPT1 pathology and exhibit aberrant protein palmitoylation. Acta Neuropathol. 2016 Apr; 131(4):621-37.
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Dawson G, Schroeder C, Dawson PE. Palmitoyl:protein thioesterase (PPT1) inhibitors can act as pharmacological chaperones in infantile Batten disease. Biochem Biophys Res Commun. 2010 Apr 23; 395(1):66-9.
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Elkay M, Silver K, Penn RD, Dalvi A. Dystonic storm due to Batten's disease treated with pallidotomy and deep brain stimulation. Mov Disord. 2009 May 15; 24(7):1048-53.
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Qin J, Goswami R, Dawson S, Dawson G. Expression of the receptor for advanced glycation end products in oligodendrocytes in response to oxidative stress. J Neurosci Res. 2008 Aug 15; 86(11):2414-22.
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Hobert JA, Dawson G. A novel role of the Batten disease gene CLN3: association with BMP synthesis. Biochem Biophys Res Commun. 2007 Jun 22; 358(1):111-6.
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Hobert JA, Dawson G. Neuronal ceroid lipofuscinoses therapeutic strategies: past, present and future. Biochim Biophys Acta. 2006 Oct; 1762(10):945-53.
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Cho S, Dawson PE, Dawson G. Role of palmitoyl-protein thioesterase in cell death: implications for infantile neuronal ceroid lipofuscinosis. Eur J Paediatr Neurol. 2001; 5 Suppl A:53-5.
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Cho S, Dawson PE, Dawson G. Antisense palmitoyl protein thioesterase 1 (PPT1) treatment inhibits PPT1 activity and increases cell death in LA-N-5 neuroblastoma cells. J Neurosci Res. 2000 Oct 15; 62(2):234-40.
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