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

Aging Research Center Home Page All Previous Aging Related Articles On-line Medical Dictionary National Library of Medicine's PubMed directory of MEDLINE citations. A Mouse Model for Finnish Variant Late Infantile Neuronal Ceroid Lipofuscinosis, CLN5, reveals neuropathology associated with early aging. - 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

HOME HELP FEEDBACK SUBSCRIPTIONS ... SEARCH QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This item requires a subscription to Journal of Neuroscience Online. Full Text A Mouse Model of Classical Late-Infantile Neuronal Ceroid Lipofuscinosis Based on... Sleat et al. J. Neurosci.. 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: ISI Web of Science (2) PubMed PubMed Citation Articles by Sleat, D. E. Articles by Lobel, P. To view this item, select one of the options below: Sign In User Name Sign in without cookies. Can't get past this page? Help with Cookies. Need to Activate? Password Forgot your user name or password? Sign Up Subscribe to the Journal - Subscribe to the print and/or online journal. HOME HELP FEEDBACK SUBSCRIPTIONS ... SEARCH

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

JOURNAL HOME HELP FEEDBACK SUBSCRIPTIONS ... SEARCH QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: Journal of Heredity Advance Access published online on June 15, 2005 Journal of Heredity, doi:10.1093/jhered/esi088 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. Article Characterization of Candidate Genes for Neuronal Ceroid Lipofuscinosis in Dog and O. Distl To whom correspondence should be addressed. 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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