Changes of copper level and cytochrome c oxidase activity in the macular mouse with age
The macular mouse is an animal model of Menkes disease. The neurological degeneration is caused by decreased cuproenzymes activity, such as cytochrome c oxidase (CCO), associated with copper deficiency in the brain. We investigated the age-related changes in copper concentration and CCO activity in...
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| Veröffentlicht in: | Brain & development (Tokyo. 1979) 1991-05, Vol.13 (3), p.184-186 |
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| container_title | Brain & development (Tokyo. 1979) |
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| creator | Meguro, Yuko Kodama, Hiroko Abe, Toshiaki Kobayashi, Shigeichi Kodama, Yukio Nishimura, Masahiko |
| description | The macular mouse is an animal model of Menkes disease. The neurological degeneration is caused by decreased cuproenzymes activity, such as cytochrome
c oxidase (CCO), associated with copper deficiency in the brain. We investigated the age-related changes in copper concentration and CCO activity in the brain of macular mice which were given a single injection of cupric chloride on postnatal day 7. The copper concentration in the brain of macular mice was always about 40% of that of the age-matched controls. However, the copper concentration of both macular and control mice increased with age gradually. The CCO activity in the brain of macular mice was significantly lower than that of controls at the age of 8 days. However the activity in macular mice increased with growth and reached a level equal to the controls at 180 days. These results suggest that the improvement of CCO activity in the brain of macular mice is due to the brain copper concentration which increased with age. Therefore, parenteral administration of copper is recommended especially during infancy in patients with Menkes disease. |
| doi_str_mv | 10.1016/S0387-7604(12)80027-1 |
| format | Article |
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c oxidase (CCO), associated with copper deficiency in the brain. We investigated the age-related changes in copper concentration and CCO activity in the brain of macular mice which were given a single injection of cupric chloride on postnatal day 7. The copper concentration in the brain of macular mice was always about 40% of that of the age-matched controls. However, the copper concentration of both macular and control mice increased with age gradually. The CCO activity in the brain of macular mice was significantly lower than that of controls at the age of 8 days. However the activity in macular mice increased with growth and reached a level equal to the controls at 180 days. These results suggest that the improvement of CCO activity in the brain of macular mice is due to the brain copper concentration which increased with age. Therefore, parenteral administration of copper is recommended especially during infancy in patients with Menkes disease.</description><identifier>ISSN: 0387-7604</identifier><identifier>EISSN: 1872-7131</identifier><identifier>DOI: 10.1016/S0387-7604(12)80027-1</identifier><identifier>PMID: 1656807</identifier><identifier>CODEN: NTHAA7</identifier><language>eng</language><publisher>Amsterdam: Elsevier B.V</publisher><subject>Aging - metabolism ; Animals ; Biological and medical sciences ; brain ; Brain - enzymology ; Copper - blood ; copper concentration ; cytochrome c oxidase ; Disease Models, Animal ; Electron Transport Complex IV - metabolism ; macular mice ; Male ; Medical sciences ; Menkes disease ; Menkes Kinky Hair Syndrome - enzymology ; Menkes Kinky Hair Syndrome - metabolism ; Metabolic diseases ; Mice ; Mice, Neurologic Mutants</subject><ispartof>Brain & development (Tokyo. 1979), 1991-05, Vol.13 (3), p.184-186</ispartof><rights>1991</rights><rights>1992 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c389t-610cd468ecf34aef27c469da2b1467c37d36ce165b9d8f07871da189b8e273313</citedby><cites>FETCH-LOGICAL-c389t-610cd468ecf34aef27c469da2b1467c37d36ce165b9d8f07871da189b8e273313</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/S0387-7604(12)80027-1$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3548,27922,27923,45993</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=5423237$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/1656807$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Meguro, Yuko</creatorcontrib><creatorcontrib>Kodama, Hiroko</creatorcontrib><creatorcontrib>Abe, Toshiaki</creatorcontrib><creatorcontrib>Kobayashi, Shigeichi</creatorcontrib><creatorcontrib>Kodama, Yukio</creatorcontrib><creatorcontrib>Nishimura, Masahiko</creatorcontrib><title>Changes of copper level and cytochrome c oxidase activity in the macular mouse with age</title><title>Brain & development (Tokyo. 1979)</title><addtitle>Brain Dev</addtitle><description>The macular mouse is an animal model of Menkes disease. The neurological degeneration is caused by decreased cuproenzymes activity, such as cytochrome
c oxidase (CCO), associated with copper deficiency in the brain. We investigated the age-related changes in copper concentration and CCO activity in the brain of macular mice which were given a single injection of cupric chloride on postnatal day 7. The copper concentration in the brain of macular mice was always about 40% of that of the age-matched controls. However, the copper concentration of both macular and control mice increased with age gradually. The CCO activity in the brain of macular mice was significantly lower than that of controls at the age of 8 days. However the activity in macular mice increased with growth and reached a level equal to the controls at 180 days. These results suggest that the improvement of CCO activity in the brain of macular mice is due to the brain copper concentration which increased with age. Therefore, parenteral administration of copper is recommended especially during infancy in patients with Menkes disease.</description><subject>Aging - metabolism</subject><subject>Animals</subject><subject>Biological and medical sciences</subject><subject>brain</subject><subject>Brain - enzymology</subject><subject>Copper - blood</subject><subject>copper concentration</subject><subject>cytochrome c oxidase</subject><subject>Disease Models, Animal</subject><subject>Electron Transport Complex IV - metabolism</subject><subject>macular mice</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Menkes disease</subject><subject>Menkes Kinky Hair Syndrome - enzymology</subject><subject>Menkes Kinky Hair Syndrome - metabolism</subject><subject>Metabolic diseases</subject><subject>Mice</subject><subject>Mice, Neurologic Mutants</subject><issn>0387-7604</issn><issn>1872-7131</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1991</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkE1vEzEQhi0EatPAT6jkQ4XoYcFjb2znVKGIfkiVOADiaDnj2cbV7jq1N4H8ezZN1B45zeF9ZubVw9g5iM8gQH_5IZQ1ldGi_gTy0gohTQVv2ASskZUBBW_Z5AU5ZWelPAohQII4YSegZ9oKM2G_FyvfP1DhqeGY1mvKvKUttdz3geNuSLjKqSOOPP2NwRfiHoe4jcOOx54PK-Kdx03rM-_SZkz_xGHF_QO9Z-8a3xb6cJxT9uv628_FbXX__eZu8fW-QmXnQ6VBYKi1JWxU7amRBms9D14uodYGlQlKI41tl_NgG2GsgeDBzpeWpFEK1JR9PNxd5_S0oTK4LhaktvU9jYWckaDBKj2CswOIOZWSqXHrHDufdw6E2wt1z0Ld3pYD6Z6Fuv2D8-ODzbKj8Lp1MDjmF8fcF_Rtk32Psbxgs1oqqfbY1QGjUcY2UnYFI_VIIWbCwYUU_1PkH8XskYY</recordid><startdate>199105</startdate><enddate>199105</enddate><creator>Meguro, Yuko</creator><creator>Kodama, Hiroko</creator><creator>Abe, Toshiaki</creator><creator>Kobayashi, Shigeichi</creator><creator>Kodama, Yukio</creator><creator>Nishimura, Masahiko</creator><general>Elsevier B.V</general><general>Elsevier Science</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>7X8</scope><scope>8BM</scope></search><sort><creationdate>199105</creationdate><title>Changes of copper level and cytochrome c oxidase activity in the macular mouse with age</title><author>Meguro, Yuko ; Kodama, Hiroko ; Abe, Toshiaki ; Kobayashi, Shigeichi ; Kodama, Yukio ; Nishimura, Masahiko</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c389t-610cd468ecf34aef27c469da2b1467c37d36ce165b9d8f07871da189b8e273313</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1991</creationdate><topic>Aging - metabolism</topic><topic>Animals</topic><topic>Biological and medical sciences</topic><topic>brain</topic><topic>Brain - enzymology</topic><topic>Copper - blood</topic><topic>copper concentration</topic><topic>cytochrome c oxidase</topic><topic>Disease Models, Animal</topic><topic>Electron Transport Complex IV - metabolism</topic><topic>macular mice</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Menkes disease</topic><topic>Menkes Kinky Hair Syndrome - enzymology</topic><topic>Menkes Kinky Hair Syndrome - metabolism</topic><topic>Metabolic diseases</topic><topic>Mice</topic><topic>Mice, Neurologic Mutants</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Meguro, Yuko</creatorcontrib><creatorcontrib>Kodama, Hiroko</creatorcontrib><creatorcontrib>Abe, Toshiaki</creatorcontrib><creatorcontrib>Kobayashi, Shigeichi</creatorcontrib><creatorcontrib>Kodama, Yukio</creatorcontrib><creatorcontrib>Nishimura, Masahiko</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>MEDLINE - Academic</collection><collection>ComDisDome</collection><jtitle>Brain & development (Tokyo. 1979)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Meguro, Yuko</au><au>Kodama, Hiroko</au><au>Abe, Toshiaki</au><au>Kobayashi, Shigeichi</au><au>Kodama, Yukio</au><au>Nishimura, Masahiko</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Changes of copper level and cytochrome c oxidase activity in the macular mouse with age</atitle><jtitle>Brain & development (Tokyo. 1979)</jtitle><addtitle>Brain Dev</addtitle><date>1991-05</date><risdate>1991</risdate><volume>13</volume><issue>3</issue><spage>184</spage><epage>186</epage><pages>184-186</pages><issn>0387-7604</issn><eissn>1872-7131</eissn><coden>NTHAA7</coden><abstract>The macular mouse is an animal model of Menkes disease. The neurological degeneration is caused by decreased cuproenzymes activity, such as cytochrome
c oxidase (CCO), associated with copper deficiency in the brain. We investigated the age-related changes in copper concentration and CCO activity in the brain of macular mice which were given a single injection of cupric chloride on postnatal day 7. The copper concentration in the brain of macular mice was always about 40% of that of the age-matched controls. However, the copper concentration of both macular and control mice increased with age gradually. The CCO activity in the brain of macular mice was significantly lower than that of controls at the age of 8 days. However the activity in macular mice increased with growth and reached a level equal to the controls at 180 days. These results suggest that the improvement of CCO activity in the brain of macular mice is due to the brain copper concentration which increased with age. Therefore, parenteral administration of copper is recommended especially during infancy in patients with Menkes disease.</abstract><cop>Amsterdam</cop><pub>Elsevier B.V</pub><pmid>1656807</pmid><doi>10.1016/S0387-7604(12)80027-1</doi><tpages>3</tpages></addata></record> |
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| source | MEDLINE; ScienceDirect Journals (5 years ago - present) |
| subjects | Aging - metabolism Animals Biological and medical sciences brain Brain - enzymology Copper - blood copper concentration cytochrome c oxidase Disease Models, Animal Electron Transport Complex IV - metabolism macular mice Male Medical sciences Menkes disease Menkes Kinky Hair Syndrome - enzymology Menkes Kinky Hair Syndrome - metabolism Metabolic diseases Mice Mice, Neurologic Mutants |
| title | Changes of copper level and cytochrome c oxidase activity in the macular mouse with age |
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