The chemical biology of branched-chain lipid metabolism.
Mammalian metabolism of some lipids including 3-methyl and 2-methyl branched-chain fatty acids occurs within peroxisomes. Such lipids, including phytanic and pristanic acids, are commonly found within the human diet and may be derived from chlorophyll in plant extracts. Due to the presence of a meth...
| Main Authors: | , , , , , |
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| Format: | Journal article |
| Language: | English |
| Published: |
2003
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| _version_ | 1797070364728098816 |
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| author | Mukherji, M Schofield, C Wierzbicki, A Jansen, G Wanders, R Lloyd, MD |
| author_facet | Mukherji, M Schofield, C Wierzbicki, A Jansen, G Wanders, R Lloyd, MD |
| author_sort | Mukherji, M |
| collection | OXFORD |
| description | Mammalian metabolism of some lipids including 3-methyl and 2-methyl branched-chain fatty acids occurs within peroxisomes. Such lipids, including phytanic and pristanic acids, are commonly found within the human diet and may be derived from chlorophyll in plant extracts. Due to the presence of a methyl group at its beta-carbon, the well-characterised beta-oxidation pathway cannot degrade phytanic acid. Instead its alpha-methylene group is oxidatively excised to give pristanic acid, which can be metabolised by the beta-oxidation pathway. Many defects in the alpha-oxidation pathway result in an accumulation of phytanic acid, leading to neurological distress, deterioration of vision, deafness, loss of coordination and eventual death. Details of the alpha-oxidation pathway have only recently been elucidated, and considerable progress has been made in understanding the detailed enzymology of one of the oxidative steps within this pathway. This review summarises these recent advances and considers the roles and likely mechanisms of the enzymes within the alpha-oxidation pathway. |
| first_indexed | 2024-03-06T22:37:45Z |
| format | Journal article |
| id | oxford-uuid:5a811543-124f-4bbd-953c-87fe31a04f77 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-06T22:37:45Z |
| publishDate | 2003 |
| record_format | dspace |
| spelling | oxford-uuid:5a811543-124f-4bbd-953c-87fe31a04f772022-03-26T17:16:08ZThe chemical biology of branched-chain lipid metabolism.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:5a811543-124f-4bbd-953c-87fe31a04f77EnglishSymplectic Elements at Oxford2003Mukherji, MSchofield, CWierzbicki, AJansen, GWanders, RLloyd, MDMammalian metabolism of some lipids including 3-methyl and 2-methyl branched-chain fatty acids occurs within peroxisomes. Such lipids, including phytanic and pristanic acids, are commonly found within the human diet and may be derived from chlorophyll in plant extracts. Due to the presence of a methyl group at its beta-carbon, the well-characterised beta-oxidation pathway cannot degrade phytanic acid. Instead its alpha-methylene group is oxidatively excised to give pristanic acid, which can be metabolised by the beta-oxidation pathway. Many defects in the alpha-oxidation pathway result in an accumulation of phytanic acid, leading to neurological distress, deterioration of vision, deafness, loss of coordination and eventual death. Details of the alpha-oxidation pathway have only recently been elucidated, and considerable progress has been made in understanding the detailed enzymology of one of the oxidative steps within this pathway. This review summarises these recent advances and considers the roles and likely mechanisms of the enzymes within the alpha-oxidation pathway. |
| spellingShingle | Mukherji, M Schofield, C Wierzbicki, A Jansen, G Wanders, R Lloyd, MD The chemical biology of branched-chain lipid metabolism. |
| title | The chemical biology of branched-chain lipid metabolism. |
| title_full | The chemical biology of branched-chain lipid metabolism. |
| title_fullStr | The chemical biology of branched-chain lipid metabolism. |
| title_full_unstemmed | The chemical biology of branched-chain lipid metabolism. |
| title_short | The chemical biology of branched-chain lipid metabolism. |
| title_sort | chemical biology of branched chain lipid metabolism |
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