Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis)
Immunochemical studies demonstrate that subunit c of mitochondrial ATP synthase is stored in the late-infantile, juvenile and adult forms of Batten's disease. It does not accumulate in the infantile form, or in other conditions involving lysosomal hypertrophy. These results suggest that the def...
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| Veröffentlicht in: | Biochemical journal 1991-04, Vol.275 (1), p.269-272 |
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| container_title | Biochemical journal |
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| creator | HALL, N. A LAKE, B. D DEWJI, N. N PATRICK, A. D |
| description | Immunochemical studies demonstrate that subunit c of mitochondrial ATP synthase is stored in the late-infantile, juvenile and adult forms of Batten's disease. It does not accumulate in the infantile form, or in other conditions involving lysosomal hypertrophy. These results suggest that the defective metabolism of subunit c is central to the pathogenesis of these three forms of Batten's disease. |
| doi_str_mv | 10.1042/bj2750269 |
| format | Article |
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These results suggest that the defective metabolism of subunit c is central to the pathogenesis of these three forms of Batten's disease.</description><subject>Aged</subject><subject>Aging - metabolism</subject><subject>Biological and medical sciences</subject><subject>Blotting, Western</subject><subject>Brain - enzymology</subject><subject>Brain - ultrastructure</subject><subject>Errors of metabolism</subject><subject>H super(+)-transporting ATP synthase</subject><subject>Humans</subject><subject>Immunoenzyme Techniques</subject><subject>lysosomes</subject><subject>Lysosomes - enzymology</subject><subject>Macromolecular Substances</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Middle Aged</subject><subject>Miscellaneous hereditary metabolic disorders</subject><subject>Mitochondria - enzymology</subject><subject>Molecular Weight</subject><subject>Neuronal Ceroid-Lipofuscinoses - enzymology</subject><subject>Proton-Translocating ATPases - metabolism</subject><subject>Submitochondrial Particles - enzymology</subject><issn>0264-6021</issn><issn>1470-8728</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1991</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkU1r3DAQhkVpSbdpD_0BBV-SJge3I1m25UsgCU1aWGgP6VmMZSmrYEsbjR3Yfx9vd8nHqScx8z56GHgZ-8zhGwcpvrd3oi5BVM0btuCyhlzVQr1li3kl8woEf88-EN0BcAkSDtgBV6JSRbFgq-WGIsUB-4zGmPDWZtFlNLVT8GNmtsPgx2hWMXTJz9T5zZ-MNmFcIdnMh-wCx9GGr5R1nux2d2Jsir7Le7-ObiLjQyRPpx_ZO4c92U_795D9vfpxc_kzX_6-_nV5vsyN5KLJraq7UghhDVdOuM4ZLBtEh7WStTB12woFUjpsC9MaURlVAi8kQOvA8RKKQ3a2866ndrCdsWFM2Ot18gOmjY7o9esk-JW-jQ-az79n8yw43gtSvJ8sjXrwZGzfY7BxIq2g5I2U5X9BXnEQ8p_xdAeaFImSdU_XcNDb_vRTfzP75eX5z-SusDk_2udIBnuXMBhPz1hTVQJkVTwCH3CkMg</recordid><startdate>19910401</startdate><enddate>19910401</enddate><creator>HALL, N. 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A</creatorcontrib><creatorcontrib>LAKE, B. D</creatorcontrib><creatorcontrib>DEWJI, N. N</creatorcontrib><creatorcontrib>PATRICK, A. D</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>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>Biochemistry Abstracts 1</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Biochemical journal</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>HALL, N. A</au><au>LAKE, B. D</au><au>DEWJI, N. N</au><au>PATRICK, A. D</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis)</atitle><jtitle>Biochemical journal</jtitle><addtitle>Biochem J</addtitle><date>1991-04-01</date><risdate>1991</risdate><volume>275</volume><issue>1</issue><spage>269</spage><epage>272</epage><pages>269-272</pages><issn>0264-6021</issn><eissn>1470-8728</eissn><abstract>Immunochemical studies demonstrate that subunit c of mitochondrial ATP synthase is stored in the late-infantile, juvenile and adult forms of Batten's disease. It does not accumulate in the infantile form, or in other conditions involving lysosomal hypertrophy. These results suggest that the defective metabolism of subunit c is central to the pathogenesis of these three forms of Batten's disease.</abstract><cop>Colchester</cop><pub>Portland Press</pub><pmid>1826833</pmid><doi>10.1042/bj2750269</doi><tpages>4</tpages><oa>free_for_read</oa></addata></record> |
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| ispartof | Biochemical journal, 1991-04, Vol.275 (1), p.269-272 |
| issn | 0264-6021 1470-8728 |
| language | eng |
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| source | MEDLINE; EZB-FREE-00999 freely available EZB journals; PubMed Central; Alma/SFX Local Collection |
| subjects | Aged Aging - metabolism Biological and medical sciences Blotting, Western Brain - enzymology Brain - ultrastructure Errors of metabolism H super(+)-transporting ATP synthase Humans Immunoenzyme Techniques lysosomes Lysosomes - enzymology Macromolecular Substances Medical sciences Metabolic diseases Middle Aged Miscellaneous hereditary metabolic disorders Mitochondria - enzymology Molecular Weight Neuronal Ceroid-Lipofuscinoses - enzymology Proton-Translocating ATPases - metabolism Submitochondrial Particles - enzymology |
| title | Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis) |
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