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ATP-binding cassette sub-family D member 1 ATP-binding cassette sub-family D member 2 Very long-chain acyl-CoA synthetase 2- hydroxyacyl- CoA lyase 1 Fatty aldehyde dehydrogenase Phytanoyl-CoA dioxygenase, peroxisomal Pristanic acid Acetyl-CoA Propionyl-CoA Isobutyryl-CoA Phytanoyl-CoA Phytanoyl-CoA Accumulation Phytanic acid ATP CoA ADP PPi 2-Hydroxyphytanoyl-CoA Pristanal Formyl-CoA NAD H2O NADH Oxoglutaric acid O2 Succinic acid CO2 Beta Oxidation Phytanic Acid Peroxisomal Oxidation Iron Magnesium Thiamine pyrophosphate Ascorbic acid Peroxisome Intracellular Space Extracellular Space
Peroxisome Unknown Unknown SLC27A2 HACL1 ALDH3A2 PHYH Pristanic acid Acetyl-CoA Propionyl-CoA Isobutyryl-CoA Phytanoyl-CoA Phytanoyl-CoA Accumulation Phytanic acid Adenosine triphosphate Coenzyme A Adenosine diphosphate Pyrophosphate 2- Hydroxyphytanoyl- CoA Pristanal Formyl-CoA NAD Water NADH Oxoglutaric acid Oxygen Succinic acid Carbon dioxide Beta Oxidation Phytanic Acid Peroxisomal Oxidation
SLC27A2 HACL1 ALDH3A2 PHYH Pristan Ac-CoA PropCoA IsoBCoA PhytCoA PhytCoA Accumulation Phytana ATP CoA ADP PPi 2HPCoA Pristal FormCoA NAD H2O NADH AKG O2 Succini CO2 Bet Oxi PW000041 Iron Mg2+ ThiamPP VitC Peroxisome Intracellular Space Extracellular Space
Peroxisome SLC27A2 HACL1 ALDH3A2 PHYH Pristan Ac-CoA PropCoA IsoBCoA PhytCoA PhytCoA Accumulation Phytana ATP CoA ADP Ppi 2HPCoA Pristal FormCoA NAD H2O NADH AKG O2 Succini CO2 Bet Oxi PW000041