Early changes in gene expression in two models of Batten disease
Infantile and juvenile neuronal ceroid lipofuscinosis (NCLs) are progressive neurodegenerative disorders of childhood with distinct ages of clinical onset, but with a similar pathological outcome. Infantile and juvenile NCL are inherited in an autosomal recessive manner due to mutations in the CLN1...
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| Veröffentlicht in: | FEBS letters 2003-03, Vol.538 (1-3), p.207-212 |
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| container_title | FEBS letters |
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| creator | Elshatory, Yasser Brooks, Andrew I. Chattopadhyay, Subrata Curran, Timothy M. Gupta, Praveena Ramalingam, Vijay Hofmann, Sandra L. Pearce, David A. |
| description | Infantile and juvenile neuronal ceroid lipofuscinosis (NCLs) are progressive neurodegenerative disorders of childhood with distinct ages of clinical onset, but with a similar pathological outcome. Infantile and juvenile NCL are inherited in an autosomal recessive manner due to mutations in the CLN1 and CLN3 genes, respectively. Recently developed Cln1- and Cln3-knockout mouse models share similarities in pathology with the respective human disease. Using oligonucleotide arrays we identified reproducible changes in gene expression in the brains of both 10-week-old Cln1- and Cln3-knockout mice as compared to wild-type controls, and confirmed changes in levels of several of the cognate proteins by immunoblotting. Despite the similarities in pathology, the two mutations affect the expression of different, non-overlapping sets of genes. The possible significance of these changes and the pathological mechanisms underlying NCL diseases are discussed. |
| doi_str_mv | 10.1016/S0014-5793(03)00162-5 |
| format | Article |
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Infantile and juvenile NCL are inherited in an autosomal recessive manner due to mutations in the CLN1 and CLN3 genes, respectively. Recently developed Cln1- and Cln3-knockout mouse models share similarities in pathology with the respective human disease. Using oligonucleotide arrays we identified reproducible changes in gene expression in the brains of both 10-week-old Cln1- and Cln3-knockout mice as compared to wild-type controls, and confirmed changes in levels of several of the cognate proteins by immunoblotting. Despite the similarities in pathology, the two mutations affect the expression of different, non-overlapping sets of genes. The possible significance of these changes and the pathological mechanisms underlying NCL diseases are discussed.</description><identifier>ISSN: 0014-5793</identifier><identifier>EISSN: 1873-3468</identifier><identifier>DOI: 10.1016/S0014-5793(03)00162-5</identifier><identifier>PMID: 12633880</identifier><language>eng</language><publisher>England: Elsevier B.V</publisher><subject>Animals ; Batten disease ; Brain - metabolism ; CLN1 ; CLN3 ; Gene expression ; Gene Expression Profiling ; Gene Expression Regulation ; Humans ; Membrane Glycoproteins ; Membrane Proteins - genetics ; Membrane Proteins - physiology ; Mice ; Mice, Knockout ; Microarray ; Molecular Chaperones ; Neurodegeneration ; Neuronal ceroid lipofuscinose ; Neuronal Ceroid-Lipofuscinoses - genetics ; Palmitoyl-protein thioesterase-1 ; Proteins - genetics ; Proteins - physiology ; Thiolester Hydrolases</subject><ispartof>FEBS letters, 2003-03, Vol.538 (1-3), p.207-212</ispartof><rights>2003</rights><rights>FEBS Letters 538 (2003) 1873-3468 © 2015 Federation of European Biochemical Societies</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4265-1ef98f843ac7f018120bd9732915adbafaa53f8866b8c7b527392f2eba7d1ab03</citedby><cites>FETCH-LOGICAL-c4265-1ef98f843ac7f018120bd9732915adbafaa53f8866b8c7b527392f2eba7d1ab03</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1016%2FS0014-5793%2803%2900162-5$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S0014579303001625$$EHTML$$P50$$Gelsevier$$Hfree_for_read</linktohtml><link.rule.ids>314,776,780,1411,1427,3537,27901,27902,45550,45551,46384,46808,65306</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/12633880$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Elshatory, Yasser</creatorcontrib><creatorcontrib>Brooks, Andrew I.</creatorcontrib><creatorcontrib>Chattopadhyay, Subrata</creatorcontrib><creatorcontrib>Curran, Timothy M.</creatorcontrib><creatorcontrib>Gupta, Praveena</creatorcontrib><creatorcontrib>Ramalingam, Vijay</creatorcontrib><creatorcontrib>Hofmann, Sandra L.</creatorcontrib><creatorcontrib>Pearce, David A.</creatorcontrib><title>Early changes in gene expression in two models of Batten disease</title><title>FEBS letters</title><addtitle>FEBS Lett</addtitle><description>Infantile and juvenile neuronal ceroid lipofuscinosis (NCLs) are progressive neurodegenerative disorders of childhood with distinct ages of clinical onset, but with a similar pathological outcome. Infantile and juvenile NCL are inherited in an autosomal recessive manner due to mutations in the CLN1 and CLN3 genes, respectively. Recently developed Cln1- and Cln3-knockout mouse models share similarities in pathology with the respective human disease. Using oligonucleotide arrays we identified reproducible changes in gene expression in the brains of both 10-week-old Cln1- and Cln3-knockout mice as compared to wild-type controls, and confirmed changes in levels of several of the cognate proteins by immunoblotting. Despite the similarities in pathology, the two mutations affect the expression of different, non-overlapping sets of genes. The possible significance of these changes and the pathological mechanisms underlying NCL diseases are discussed.</description><subject>Animals</subject><subject>Batten disease</subject><subject>Brain - metabolism</subject><subject>CLN1</subject><subject>CLN3</subject><subject>Gene expression</subject><subject>Gene Expression Profiling</subject><subject>Gene Expression Regulation</subject><subject>Humans</subject><subject>Membrane Glycoproteins</subject><subject>Membrane Proteins - genetics</subject><subject>Membrane Proteins - physiology</subject><subject>Mice</subject><subject>Mice, Knockout</subject><subject>Microarray</subject><subject>Molecular Chaperones</subject><subject>Neurodegeneration</subject><subject>Neuronal ceroid lipofuscinose</subject><subject>Neuronal Ceroid-Lipofuscinoses - genetics</subject><subject>Palmitoyl-protein thioesterase-1</subject><subject>Proteins - genetics</subject><subject>Proteins - physiology</subject><subject>Thiolester Hydrolases</subject><issn>0014-5793</issn><issn>1873-3468</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2003</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkE1PwzAMhiMEYmPwE0A9ITgU8rEm6QnYtAHSJA7AOUpTZwR17Ug6xv497TrBESRLlu3Xr60HoVOCrwgm_PoZYzKME5GyC8wum4LTONlDfSIFi9mQy33U_5H00FEI77ipJUkPUY9QzpiUuI9uJ9oXm8i86XIOIXJlNIcSIvhaegjBVWXbqtdVtKhyKEJU2Wik6xrKKHcBdIBjdGB1EeBklwfodTp5GT_Es6f7x_HdLDZDypOYgE2llUOmjbDtGxRneSoYTUmi80xbrRNmpeQ8k0ZkCRUspZZCpkVOdIbZAJ13vktffawg1GrhgoGi0CVUq6AEw5ILnjbCpBMaX4XgwaqldwvtN4pg1aJTW3Sq5aJwEy06lTR7Z7sDq2wB-e_WjlUjeOgEa1fA5n-uajoZ0e2kHWC2bbe3bjqrBil8OvAqGAelgdx5MLXKK_fHt99xjJDM</recordid><startdate>20030313</startdate><enddate>20030313</enddate><creator>Elshatory, Yasser</creator><creator>Brooks, Andrew I.</creator><creator>Chattopadhyay, Subrata</creator><creator>Curran, Timothy M.</creator><creator>Gupta, Praveena</creator><creator>Ramalingam, Vijay</creator><creator>Hofmann, Sandra L.</creator><creator>Pearce, David A.</creator><general>Elsevier B.V</general><scope>6I.</scope><scope>AAFTH</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20030313</creationdate><title>Early changes in gene expression in two models of Batten disease</title><author>Elshatory, Yasser ; Brooks, Andrew I. ; Chattopadhyay, Subrata ; Curran, Timothy M. ; Gupta, Praveena ; Ramalingam, Vijay ; Hofmann, Sandra L. ; Pearce, David A.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4265-1ef98f843ac7f018120bd9732915adbafaa53f8866b8c7b527392f2eba7d1ab03</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2003</creationdate><topic>Animals</topic><topic>Batten disease</topic><topic>Brain - metabolism</topic><topic>CLN1</topic><topic>CLN3</topic><topic>Gene expression</topic><topic>Gene Expression Profiling</topic><topic>Gene Expression Regulation</topic><topic>Humans</topic><topic>Membrane Glycoproteins</topic><topic>Membrane Proteins - genetics</topic><topic>Membrane Proteins - physiology</topic><topic>Mice</topic><topic>Mice, Knockout</topic><topic>Microarray</topic><topic>Molecular Chaperones</topic><topic>Neurodegeneration</topic><topic>Neuronal ceroid lipofuscinose</topic><topic>Neuronal Ceroid-Lipofuscinoses - genetics</topic><topic>Palmitoyl-protein thioesterase-1</topic><topic>Proteins - genetics</topic><topic>Proteins - physiology</topic><topic>Thiolester Hydrolases</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Elshatory, Yasser</creatorcontrib><creatorcontrib>Brooks, Andrew I.</creatorcontrib><creatorcontrib>Chattopadhyay, Subrata</creatorcontrib><creatorcontrib>Curran, Timothy M.</creatorcontrib><creatorcontrib>Gupta, Praveena</creatorcontrib><creatorcontrib>Ramalingam, Vijay</creatorcontrib><creatorcontrib>Hofmann, Sandra L.</creatorcontrib><creatorcontrib>Pearce, David A.</creatorcontrib><collection>ScienceDirect Open Access Titles</collection><collection>Elsevier:ScienceDirect:Open Access</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>FEBS letters</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Elshatory, Yasser</au><au>Brooks, Andrew I.</au><au>Chattopadhyay, Subrata</au><au>Curran, Timothy M.</au><au>Gupta, Praveena</au><au>Ramalingam, Vijay</au><au>Hofmann, Sandra L.</au><au>Pearce, David A.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Early changes in gene expression in two models of Batten disease</atitle><jtitle>FEBS letters</jtitle><addtitle>FEBS Lett</addtitle><date>2003-03-13</date><risdate>2003</risdate><volume>538</volume><issue>1-3</issue><spage>207</spage><epage>212</epage><pages>207-212</pages><issn>0014-5793</issn><eissn>1873-3468</eissn><abstract>Infantile and juvenile neuronal ceroid lipofuscinosis (NCLs) are progressive neurodegenerative disorders of childhood with distinct ages of clinical onset, but with a similar pathological outcome. Infantile and juvenile NCL are inherited in an autosomal recessive manner due to mutations in the CLN1 and CLN3 genes, respectively. Recently developed Cln1- and Cln3-knockout mouse models share similarities in pathology with the respective human disease. Using oligonucleotide arrays we identified reproducible changes in gene expression in the brains of both 10-week-old Cln1- and Cln3-knockout mice as compared to wild-type controls, and confirmed changes in levels of several of the cognate proteins by immunoblotting. Despite the similarities in pathology, the two mutations affect the expression of different, non-overlapping sets of genes. The possible significance of these changes and the pathological mechanisms underlying NCL diseases are discussed.</abstract><cop>England</cop><pub>Elsevier B.V</pub><pmid>12633880</pmid><doi>10.1016/S0014-5793(03)00162-5</doi><tpages>6</tpages><oa>free_for_read</oa></addata></record> |
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| source | Wiley Free Content; MEDLINE; Wiley Online Library Journals Frontfile Complete; Elsevier ScienceDirect Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Alma/SFX Local Collection |
| subjects | Animals Batten disease Brain - metabolism CLN1 CLN3 Gene expression Gene Expression Profiling Gene Expression Regulation Humans Membrane Glycoproteins Membrane Proteins - genetics Membrane Proteins - physiology Mice Mice, Knockout Microarray Molecular Chaperones Neurodegeneration Neuronal ceroid lipofuscinose Neuronal Ceroid-Lipofuscinoses - genetics Palmitoyl-protein thioesterase-1 Proteins - genetics Proteins - physiology Thiolester Hydrolases |
| title | Early changes in gene expression in two models of Batten disease |
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