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Neuronal Ceroid Lipofuscinosis:     more detail

Lysosomal Storage Diseases: Tay-Sachs Disease, Canavan Disease, Sly Syndrome, Neuronal Ceroid Lipofuscinosis, Mucopolysaccharidosis The dissection of a degenerative disease: Proceedings of four round-table conferences on the pathogenesis of Batten's disease (neuronal ceroid-lipofuscinosis) Lipofuscin and Ceroid Pigments (Advances in Experimental Medicine and Biology) Batten Disease: Diagnosis, Treatment, and Research, Volume 45 (Advances in Genetics) The Official Parent's Sourcebook on Batten Disease: A Revised and Updated Directory for the Internet Age by Icon Health Publications, 2002-11-18 Lipofuscin and Ceroid Pigments: State of the Art 1995 (Journal - Gerontology, , Vol 41, Suppl. 2) (Pt.2) Dogs help track down genes.(MEDICAL UPDATE: Cutting-edge news from a source you can trust)(Batten disease): An article from: Saturday Evening Post Batten disease: An entry from Thomson Gale's <i>Gale Encyclopedia of Neurological Disorders</i> by Michelle lee Brandt, Rosalyn, MD Carson-Dewitt, 2005 Batten disease: An entry from Thomson Gale's <i>Gale Encyclopedia of Genetic Disorders, 2nd ed.</i> by Michelle Brandt, 2005 Batten disease (SuDoc HE 20.3502:B 32) by U.S. Dept of Health and Human Services, 1992

lists with details

101. Canine Ceroid Lipofuscinosis Main Page
     http://www.caninegeneticdiseases.net/CL_site/mainCL.htm

102. A Mouse Model For Finnish Variant Late Infantile Neuronal Ceroid Lipofuscinosis,
     neuronal ceroid lipofuscinoses (NCL) comprise the most common group of childhood encephalopathies caused by mutations in eight genetic loci, CLN1CLN8.  
     http://www.arclab.org/medlineupdates/abstract_15459177.html
     
     Extractions: Aging Research Center Home Page All Previous Aging Related Articles On-line Medical Dictionary National Library of Medicine's PubMed directory of MEDLINE citations. - Kopra O, Vesa J, Von Schantz C, Manninen T, Minye H, Fabritius AL, Rapola J, Van Diggelen OP, Saarela J, Jalanko A, Peltonen L Hum Mol Genet 2004 Sep 30;. Neuronal ceroid lipofuscinoses (NCL) comprise the most common group of childhood encephalopathies caused by mutations in eight genetic loci, CLN1-CLN8. Here we have developed a novel mouse model for the human vLINCL (CLN5) by targeted deletion of exon 3 of the mouse Cln5 gene. The Cln5-/- mice showed loss of vision and accumulation of autofluorescent storage material in the central nervous system (CNS) and peripheral tissues without prominent brain atrophy. The ultrastructure of the storage material accurately replicated the abnormalities in human patients revealing mixture of lamellar profiles including fingerprint profiles as well as curvilinear and rectilinear bodies in electron microscopic analysis.

103. J. Neurosci. -- Sign In Page
     Goebel HH, Mole SE, Lake BD (1999) The neuronal ceroid lipofuscinoses (Batten disease). Washington, DC IOS. Greene ND, Bernard DL, Taschner PE, Lake BD,  
     http://www.jneurosci.org/cgi/content/full/24/41/9117
     
     Extractions: This Article Abstract Full Text (PDF) Alert me when this article is cited ... Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed ... Download to citation manager Search for citing articles in:

104. Enzyme-based Diagnosis Of Classical Late Infantile Neuronal Ceroid
     The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited neurodegenerative neuronal ceroid Lipofuscinoses Are Connected at Molecular Level  
     http://www.clinchem.org/cgi/content/full/46/7/1005

105. Characterization Of Candidate Genes For Neuronal Ceroid Lipofuscinosis In Dog --
     The neuronal ceroid lipofuscinoses (NCL) are a heterogenous group of monogenic autosomal recessive inherited progressive neurodegenerative diseases  
     http://jhered.oxfordjournals.org/cgi/content/abstract/esi088v1
     
     Extractions: This Article Full Text (PDF) Supplementary data Alert me when this article is cited ... Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Request Permissions PubMed PubMed Citation Articles by Distl, O. and O. Distl Abstract The neuronal ceroid lipofuscinoses (NCL) are a heterogenous group of monogenic autosomal recessive inherited progressive neurodegenerative diseases characterized by brain and retinal atrophy and the intracellular accumulation of autofluorescent lysosomal storage bodies resembling lipofuscin in neurons and other cells. Until today, eight forms of NCL have been classified

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