Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia
Methylmalonic acidemia is one of the most prevalent inherited metabolic disorders involving neurological deficits. In vitro experiments, animal model studies and tissue analyses from human patients suggest extensive impairment of mitochondrial energy metabolism in this disease. This review summarize...
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| Veröffentlicht in: | Journal of bioenergetics and biomembranes 2011-02, Vol.43 (1), p.39-46 |
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| creator | Melo, Daniela R Kowaltowski, Alicia J Wajner, Moacir Castilho, Roger F |
| description | Methylmalonic acidemia is one of the most prevalent inherited metabolic disorders involving neurological deficits. In vitro experiments, animal model studies and tissue analyses from human patients suggest extensive impairment of mitochondrial energy metabolism in this disease. This review summarizes changes in mitochondrial energy metabolism occurring in methylmalonic acidemia, focusing mainly on the effects of accumulated methylmalonic acid, and gives an overview of the results found in different experimental models. Overall, experiments to date suggest that mitochondrial impairment in this disease occurs through a combination of the inhibition of specific enzymes and transporters, limitation in the availability of substrates for mitochondrial metabolic pathways and oxidative damage. |
| doi_str_mv | 10.1007/s10863-011-9330-2 |
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In vitro experiments, animal model studies and tissue analyses from human patients suggest extensive impairment of mitochondrial energy metabolism in this disease. This review summarizes changes in mitochondrial energy metabolism occurring in methylmalonic acidemia, focusing mainly on the effects of accumulated methylmalonic acid, and gives an overview of the results found in different experimental models. 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In vitro experiments, animal model studies and tissue analyses from human patients suggest extensive impairment of mitochondrial energy metabolism in this disease. This review summarizes changes in mitochondrial energy metabolism occurring in methylmalonic acidemia, focusing mainly on the effects of accumulated methylmalonic acid, and gives an overview of the results found in different experimental models. 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toxicity</subject><subject>Mitochondria</subject><subject>Mitochondria - drug effects</subject><subject>Mitochondria - metabolism</subject><subject>Morphology</subject><subject>Nervous system</subject><subject>Neurodegeneration</subject><subject>Neurodegenerative Diseases - etiology</subject><subject>Neurodegenerative Diseases - metabolism</subject><subject>Organic acidemias</subject><subject>Organic Chemistry</subject><subject>Oxidative metabolism</subject><subject>Oxidative stress</subject><subject>Oxygen Consumption - drug effects</subject><subject>Oxygen Consumption - physiology</subject><subject>Tissue analysis</subject><issn>0145-479X</issn><issn>1573-6881</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2011</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNp9kE2L1TAUhoMozp3RH-BGi5tZVU-SpkmXMvgFI7MYBwQX4bQ9vTdDm4xJy3D_vSkdFVwMWSRwnvc94WHsFYd3HEC_TxxMLUvgvGykhFI8YTuutCxrY_hTtgNeqbLSzY8TdprSLQAYUPCcnQguNBcGduznNzeH7hB8Hx2OBXmK-2Mx0YxtGF2aCucLT0sMPe3XIc4u-AJTCp3Dmfri3s2HlT8cxwnH4F1XYOd6mhy-YM8GHBO9fLjP2M2nj98vvpSXV5-_Xny4LLtK1HOpRSX1IEgQDLXC1nRCqEa1mgZoQFOt1KC1aSi_sWpbQpBYC8gHDRLIM3a-9d7F8GuhNNvJpY7GET2FJdlcwlXD1Uq-_Y-8DUv0-XPWKJmtGVVniG9QF0NKkQZ7F92E8Wg52NW73bzb7N2u3q3ImdcPxUs7Uf838Ud0BsQGpDzye4r_Nj_W-mYLDRgs7qNL9uZaAJfAm0o2qpK_AVDUl5k</recordid><startdate>20110201</startdate><enddate>20110201</enddate><creator>Melo, Daniela R</creator><creator>Kowaltowski, Alicia J</creator><creator>Wajner, Moacir</creator><creator>Castilho, Roger F</creator><general>Boston : Springer US</general><general>Springer US</general><general>Springer Nature B.V</general><scope>FBQ</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>3V.</scope><scope>7QO</scope><scope>7QP</scope><scope>7TK</scope><scope>7X7</scope><scope>7XB</scope><scope>88A</scope><scope>88E</scope><scope>88I</scope><scope>8AO</scope><scope>8FD</scope><scope>8FE</scope><scope>8FG</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABJCF</scope><scope>ABUWG</scope><scope>AEUYN</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BGLVJ</scope><scope>BHPHI</scope><scope>CCPQU</scope><scope>D1I</scope><scope>DWQXO</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>KB.</scope><scope>LK8</scope><scope>M0S</scope><scope>M1P</scope><scope>M2P</scope><scope>M7P</scope><scope>P64</scope><scope>PDBOC</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>Q9U</scope></search><sort><creationdate>20110201</creationdate><title>Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia</title><author>Melo, Daniela R ; Kowaltowski, Alicia J ; Wajner, Moacir ; Castilho, Roger F</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c426t-72437f2e2e0f65ab8c22595b7ef0907e655f7789e07ea4bbea03a620202a8ae03</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2011</creationdate><topic>Amino Acid Metabolism, Inborn Errors - complications</topic><topic>Amino Acid Metabolism, Inborn Errors - metabolism</topic><topic>Animal Anatomy</topic><topic>Animal Biochemistry</topic><topic>Animal models</topic><topic>Biochemistry</topic><topic>Bioenergetics</topic><topic>Bioorganic Chemistry</topic><topic>central nervous system</topic><topic>Chemistry</topic><topic>Chemistry and Materials Science</topic><topic>Energy Metabolism - physiology</topic><topic>Histology</topic><topic>Humans</topic><topic>Metabolic disorders</topic><topic>methylmalonic acid</topic><topic>Methylmalonic Acid - metabolism</topic><topic>Methylmalonic Acid - toxicity</topic><topic>Mitochondria</topic><topic>Mitochondria - drug effects</topic><topic>Mitochondria - metabolism</topic><topic>Morphology</topic><topic>Nervous system</topic><topic>Neurodegeneration</topic><topic>Neurodegenerative Diseases - etiology</topic><topic>Neurodegenerative Diseases - metabolism</topic><topic>Organic acidemias</topic><topic>Organic Chemistry</topic><topic>Oxidative metabolism</topic><topic>Oxidative stress</topic><topic>Oxygen Consumption - 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In vitro experiments, animal model studies and tissue analyses from human patients suggest extensive impairment of mitochondrial energy metabolism in this disease. This review summarizes changes in mitochondrial energy metabolism occurring in methylmalonic acidemia, focusing mainly on the effects of accumulated methylmalonic acid, and gives an overview of the results found in different experimental models. Overall, experiments to date suggest that mitochondrial impairment in this disease occurs through a combination of the inhibition of specific enzymes and transporters, limitation in the availability of substrates for mitochondrial metabolic pathways and oxidative damage.</abstract><cop>Boston</cop><pub>Boston : Springer US</pub><pmid>21271280</pmid><doi>10.1007/s10863-011-9330-2</doi><tpages>8</tpages></addata></record> |
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| subjects | Amino Acid Metabolism, Inborn Errors - complications Amino Acid Metabolism, Inborn Errors - metabolism Animal Anatomy Animal Biochemistry Animal models Biochemistry Bioenergetics Bioorganic Chemistry central nervous system Chemistry Chemistry and Materials Science Energy Metabolism - physiology Histology Humans Metabolic disorders methylmalonic acid Methylmalonic Acid - metabolism Methylmalonic Acid - toxicity Mitochondria Mitochondria - drug effects Mitochondria - metabolism Morphology Nervous system Neurodegeneration Neurodegenerative Diseases - etiology Neurodegenerative Diseases - metabolism Organic acidemias Organic Chemistry Oxidative metabolism Oxidative stress Oxygen Consumption - drug effects Oxygen Consumption - physiology Tissue analysis |
| title | Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia |
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