The DMWD protein from the myotonic dystrophy (DM1) gene region is developmentally regulated and is present most prominently in synapse-dense brain areas
The DMWD gene is located in the myotonic dystrophy (DM1) gene cluster on 19q, just upstream of the DMPK gene. RNA and protein products of this gene are ubiquitously expressed in all adult tissues, but occur most abundant in testes and brain. Altered expression of DMWD mRNA in DM1 patients has been o...
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| Veröffentlicht in: | Brain research 2003-05, Vol.971 (1), p.116-127 |
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| creator | Westerlaken, Jolanda H.A.M Van der Zee, Catharina E.E.M Peters, Wilma Wieringa, Bé |
| description | The
DMWD gene is located in the myotonic dystrophy (DM1) gene cluster on 19q, just upstream of the
DMPK gene. RNA and protein products of this gene are ubiquitously expressed in all adult tissues, but occur most abundant in testes and brain. Altered expression of DMWD mRNA in DM1 patients has been observed, suggesting a role of the
DMWD gene products in disease manifestation. Here we focussed on DMWD expression in mouse brain and followed mRNA and protein levels and (intra)cellular location in developing brain in vivo as well as in differentiating neuronal cell cultures in vitro. In the interval between postnatal days P7 and P21, the steady-state level of DMWD mRNA remained constant, whereas the DMWD protein (doublet of 70 kDa) level gradually increased during the same period. The DMWD protein was expressed throughout the brain, at a low level in glial cells, more prominently in neurons and specifically in the neuropil of brain areas with a high density of synaptic connections. Intracellularly, DMWD was dispersed in a punctuate fashion throughout the neural cell body, the nucleus and the dendrites with their synapses, but was excluded from axons. Based on these findings and on new literature data concerning the role of
DMWD homologs in lower eukaryotes, we discuss the possible role of DMWD in the brain-related symptoms seen in DM1 patients. |
| doi_str_mv | 10.1016/S0006-8993(03)02430-2 |
| format | Article |
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DMWD gene is located in the myotonic dystrophy (DM1) gene cluster on 19q, just upstream of the
DMPK gene. RNA and protein products of this gene are ubiquitously expressed in all adult tissues, but occur most abundant in testes and brain. Altered expression of DMWD mRNA in DM1 patients has been observed, suggesting a role of the
DMWD gene products in disease manifestation. Here we focussed on DMWD expression in mouse brain and followed mRNA and protein levels and (intra)cellular location in developing brain in vivo as well as in differentiating neuronal cell cultures in vitro. In the interval between postnatal days P7 and P21, the steady-state level of DMWD mRNA remained constant, whereas the DMWD protein (doublet of 70 kDa) level gradually increased during the same period. The DMWD protein was expressed throughout the brain, at a low level in glial cells, more prominently in neurons and specifically in the neuropil of brain areas with a high density of synaptic connections. Intracellularly, DMWD was dispersed in a punctuate fashion throughout the neural cell body, the nucleus and the dendrites with their synapses, but was excluded from axons. Based on these findings and on new literature data concerning the role of
DMWD homologs in lower eukaryotes, we discuss the possible role of DMWD in the brain-related symptoms seen in DM1 patients.</description><identifier>ISSN: 0006-8993</identifier><identifier>EISSN: 1872-6240</identifier><identifier>DOI: 10.1016/S0006-8993(03)02430-2</identifier><identifier>PMID: 12691844</identifier><identifier>CODEN: BRREAP</identifier><language>eng</language><publisher>London: Elsevier B.V</publisher><subject>Animals ; Biological and medical sciences ; Blotting, Northern ; Blotting, Western ; Brain - cytology ; Brain - growth & development ; Brain - ultrastructure ; Brain Chemistry - physiology ; Cells, Cultured ; Diseases of striated muscles. Neuromuscular diseases ; DM1 patient ; DMWD brain-related symptom ; DMWD gene product ; DMWD protein ; Immunohistochemistry ; Medical sciences ; Mice ; myotonic dystrophy ; Myotonic Dystrophy - physiopathology ; Neuroglia - metabolism ; Neurology ; Neurons - metabolism ; Neurons - ultrastructure ; Neuropil - metabolism ; Postnatal DMWD expression ; Protein-Serine-Threonine Kinases - analysis ; Protein-Serine-Threonine Kinases - biosynthesis ; Proteins ; RNA, Messenger - analysis ; Synapses - metabolism ; WD repeat</subject><ispartof>Brain research, 2003-05, Vol.971 (1), p.116-127</ispartof><rights>2003 Elsevier Science B.V.</rights><rights>2003 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c488t-46edf4c4901c797ed7e9d1eb68f04177ed222325a95a30d12030b1f0937a9b23</citedby><cites>FETCH-LOGICAL-c488t-46edf4c4901c797ed7e9d1eb68f04177ed222325a95a30d12030b1f0937a9b23</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/S0006-8993(03)02430-2$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=14695363$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/12691844$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Westerlaken, Jolanda H.A.M</creatorcontrib><creatorcontrib>Van der Zee, Catharina E.E.M</creatorcontrib><creatorcontrib>Peters, Wilma</creatorcontrib><creatorcontrib>Wieringa, Bé</creatorcontrib><title>The DMWD protein from the myotonic dystrophy (DM1) gene region is developmentally regulated and is present most prominently in synapse-dense brain areas</title><title>Brain research</title><addtitle>Brain Res</addtitle><description>The
DMWD gene is located in the myotonic dystrophy (DM1) gene cluster on 19q, just upstream of the
DMPK gene. RNA and protein products of this gene are ubiquitously expressed in all adult tissues, but occur most abundant in testes and brain. Altered expression of DMWD mRNA in DM1 patients has been observed, suggesting a role of the
DMWD gene products in disease manifestation. Here we focussed on DMWD expression in mouse brain and followed mRNA and protein levels and (intra)cellular location in developing brain in vivo as well as in differentiating neuronal cell cultures in vitro. In the interval between postnatal days P7 and P21, the steady-state level of DMWD mRNA remained constant, whereas the DMWD protein (doublet of 70 kDa) level gradually increased during the same period. The DMWD protein was expressed throughout the brain, at a low level in glial cells, more prominently in neurons and specifically in the neuropil of brain areas with a high density of synaptic connections. Intracellularly, DMWD was dispersed in a punctuate fashion throughout the neural cell body, the nucleus and the dendrites with their synapses, but was excluded from axons. Based on these findings and on new literature data concerning the role of
DMWD homologs in lower eukaryotes, we discuss the possible role of DMWD in the brain-related symptoms seen in DM1 patients.</description><subject>Animals</subject><subject>Biological and medical sciences</subject><subject>Blotting, Northern</subject><subject>Blotting, Western</subject><subject>Brain - cytology</subject><subject>Brain - growth & development</subject><subject>Brain - ultrastructure</subject><subject>Brain Chemistry - physiology</subject><subject>Cells, Cultured</subject><subject>Diseases of striated muscles. Neuromuscular diseases</subject><subject>DM1 patient</subject><subject>DMWD brain-related symptom</subject><subject>DMWD gene product</subject><subject>DMWD protein</subject><subject>Immunohistochemistry</subject><subject>Medical sciences</subject><subject>Mice</subject><subject>myotonic dystrophy</subject><subject>Myotonic Dystrophy - physiopathology</subject><subject>Neuroglia - metabolism</subject><subject>Neurology</subject><subject>Neurons - metabolism</subject><subject>Neurons - ultrastructure</subject><subject>Neuropil - metabolism</subject><subject>Postnatal DMWD expression</subject><subject>Protein-Serine-Threonine Kinases - analysis</subject><subject>Protein-Serine-Threonine Kinases - biosynthesis</subject><subject>Proteins</subject><subject>RNA, Messenger - analysis</subject><subject>Synapses - metabolism</subject><subject>WD repeat</subject><issn>0006-8993</issn><issn>1872-6240</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2003</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkc9q3DAQxkVpaTZpH6FFl5bk4Eb_1rZOoWTTtJCQQxd6FLI0TlRsyZW0Ab9JH7dyd0mOAYGYmd98M8yH0AdKvlBC6_OfhJC6aqXkp4SfESY4qdgrtKJtw6qaCfIarZ6QI3Sc0u8Sci7JW3REWS1pK8QK_d0-AN7c_trgKYYMzuM-hhHnkh3nkIN3Bts55Rimhxmfbm7pGb4HDzjCvQseu4QtPMIQphF81sMwL5XdoDNYrL1dgClCKkU8hpSXMaPzJSxkmZZmr6cElQWfAHdRl5yOoNM79KbXQ4L3h_8Ebb9dbS-_Vzd31z8uv95URrRtrkQNthdGSEJNIxuwDUhLoavbngjalARjjLO1lmvNiaWMcNLRnkjeaNkxfoI-72XLXn92kLIaXTIwDNpD2CXVcFqkW_oiSFvJieCL4noPmhhSitCrKbpRx1lRohbr1H_r1OKLIuUt1qml7-NhwK4bwT53HbwqwKcDoJPRQx-1Ny49c6KWa17zwl3sOShne3QQVTIOvAHrIpisbHAvrPIPOnG3BA</recordid><startdate>20030502</startdate><enddate>20030502</enddate><creator>Westerlaken, Jolanda H.A.M</creator><creator>Van der Zee, Catharina E.E.M</creator><creator>Peters, Wilma</creator><creator>Wieringa, Bé</creator><general>Elsevier B.V</general><general>Elsevier</general><scope>IQODW</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>7TK</scope><scope>7X8</scope></search><sort><creationdate>20030502</creationdate><title>The DMWD protein from the myotonic dystrophy (DM1) gene region is developmentally regulated and is present most prominently in synapse-dense brain areas</title><author>Westerlaken, Jolanda H.A.M ; Van der Zee, Catharina E.E.M ; Peters, Wilma ; Wieringa, Bé</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c488t-46edf4c4901c797ed7e9d1eb68f04177ed222325a95a30d12030b1f0937a9b23</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2003</creationdate><topic>Animals</topic><topic>Biological and medical sciences</topic><topic>Blotting, Northern</topic><topic>Blotting, Western</topic><topic>Brain - cytology</topic><topic>Brain - growth & development</topic><topic>Brain - ultrastructure</topic><topic>Brain Chemistry - physiology</topic><topic>Cells, Cultured</topic><topic>Diseases of striated muscles. Neuromuscular diseases</topic><topic>DM1 patient</topic><topic>DMWD brain-related symptom</topic><topic>DMWD gene product</topic><topic>DMWD protein</topic><topic>Immunohistochemistry</topic><topic>Medical sciences</topic><topic>Mice</topic><topic>myotonic dystrophy</topic><topic>Myotonic Dystrophy - physiopathology</topic><topic>Neuroglia - metabolism</topic><topic>Neurology</topic><topic>Neurons - metabolism</topic><topic>Neurons - ultrastructure</topic><topic>Neuropil - metabolism</topic><topic>Postnatal DMWD expression</topic><topic>Protein-Serine-Threonine Kinases - analysis</topic><topic>Protein-Serine-Threonine Kinases - biosynthesis</topic><topic>Proteins</topic><topic>RNA, Messenger - analysis</topic><topic>Synapses - metabolism</topic><topic>WD repeat</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Westerlaken, Jolanda H.A.M</creatorcontrib><creatorcontrib>Van der Zee, Catharina E.E.M</creatorcontrib><creatorcontrib>Peters, Wilma</creatorcontrib><creatorcontrib>Wieringa, Bé</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Neurosciences Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Brain research</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Westerlaken, Jolanda H.A.M</au><au>Van der Zee, Catharina E.E.M</au><au>Peters, Wilma</au><au>Wieringa, Bé</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>The DMWD protein from the myotonic dystrophy (DM1) gene region is developmentally regulated and is present most prominently in synapse-dense brain areas</atitle><jtitle>Brain research</jtitle><addtitle>Brain Res</addtitle><date>2003-05-02</date><risdate>2003</risdate><volume>971</volume><issue>1</issue><spage>116</spage><epage>127</epage><pages>116-127</pages><issn>0006-8993</issn><eissn>1872-6240</eissn><coden>BRREAP</coden><abstract>The
DMWD gene is located in the myotonic dystrophy (DM1) gene cluster on 19q, just upstream of the
DMPK gene. RNA and protein products of this gene are ubiquitously expressed in all adult tissues, but occur most abundant in testes and brain. Altered expression of DMWD mRNA in DM1 patients has been observed, suggesting a role of the
DMWD gene products in disease manifestation. Here we focussed on DMWD expression in mouse brain and followed mRNA and protein levels and (intra)cellular location in developing brain in vivo as well as in differentiating neuronal cell cultures in vitro. In the interval between postnatal days P7 and P21, the steady-state level of DMWD mRNA remained constant, whereas the DMWD protein (doublet of 70 kDa) level gradually increased during the same period. The DMWD protein was expressed throughout the brain, at a low level in glial cells, more prominently in neurons and specifically in the neuropil of brain areas with a high density of synaptic connections. Intracellularly, DMWD was dispersed in a punctuate fashion throughout the neural cell body, the nucleus and the dendrites with their synapses, but was excluded from axons. Based on these findings and on new literature data concerning the role of
DMWD homologs in lower eukaryotes, we discuss the possible role of DMWD in the brain-related symptoms seen in DM1 patients.</abstract><cop>London</cop><cop>Amsterdam</cop><cop>New York, NY</cop><pub>Elsevier B.V</pub><pmid>12691844</pmid><doi>10.1016/S0006-8993(03)02430-2</doi><tpages>12</tpages></addata></record> |
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| ispartof | Brain research, 2003-05, Vol.971 (1), p.116-127 |
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| source | MEDLINE; Elsevier ScienceDirect Journals Complete |
| subjects | Animals Biological and medical sciences Blotting, Northern Blotting, Western Brain - cytology Brain - growth & development Brain - ultrastructure Brain Chemistry - physiology Cells, Cultured Diseases of striated muscles. Neuromuscular diseases DM1 patient DMWD brain-related symptom DMWD gene product DMWD protein Immunohistochemistry Medical sciences Mice myotonic dystrophy Myotonic Dystrophy - physiopathology Neuroglia - metabolism Neurology Neurons - metabolism Neurons - ultrastructure Neuropil - metabolism Postnatal DMWD expression Protein-Serine-Threonine Kinases - analysis Protein-Serine-Threonine Kinases - biosynthesis Proteins RNA, Messenger - analysis Synapses - metabolism WD repeat |
| title | The DMWD protein from the myotonic dystrophy (DM1) gene region is developmentally regulated and is present most prominently in synapse-dense brain areas |
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