File:Children-10-00530-g001.jpg

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Infantile Refsum Disease

Summary

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Description
English: Phytanic acid enzymatic steps of alpha-oxidation [12,13,14]. 1. On the cytosolic side of the peroxisomes, phytanic acid is activated by an acyl-CoA synthetase, resulting in phytanoyl-CoA, which is transported into the peroxisome by a specific transporter protein. 2. Phytanoyl-CoA is oxidized by phytanoyl-CoA hydroxylase, producing 2-hydroxyphytanoyl-CoA. 3. 2-hydroxyphytanoyl-CoA is then dehydrogenated by the enzyme 2-hydroxyphytanoyl-CoA lyase, resulting in pristanaldehide. 4. Pristanaldehide is oxidized by pristanal dehydrogenase, which converts it to pristanic acid.
Date
Source https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10047879/
Author Ana-Maria Slanina, Adorata-Elena Coman, Dana-Teodora Anton-Păduraru, Elena Popa, Carmen-Liliana Barbacariu, Otilia Novac, Antoneta Dacia Petroaie, Agnes-Iacinta Bacușcă, Mihaela Manole, and Adriana Cosmescu

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Date/TimeThumbnailDimensionsUserComment
current21:00, 5 November 2023Thumbnail for version as of 21:00, 5 November 2023737 × 578 (106 KB)Ozzie10aaaa (talk | contribs)Uploaded a work by Ana-Maria Slanina, Adorata-Elena Coman, Dana-Teodora Anton-Păduraru, Elena Popa, Carmen-Liliana Barbacariu, Otilia Novac, Antoneta Dacia Petroaie, Agnes-Iacinta Bacușcă, Mihaela Manole, and Adriana Cosmescu from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10047879/ with UploadWizard

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