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Mitochondria pantothenate kinase fused 4'- phosphopantothenoylcysteine decarboxylase and phosphopantothenoylcysteine synthetase phosphopantetheine adenylyltransferase Dephospho-CoA kinase CAB5 triphosphoribosyl- dephospho-CoA synthase 3-methyl-2- oxobutanoate hydroxymethyltransferase 2- dehydropantoate 2-reductase phosphopantothenate- cysteine ligase Dihydroxy-acid dehydratase, mitochondrial Pantoate-- beta-alanine ligase α- Ketoisovaleric acid Pantothenic acid ATP ADP H+ D-4'- Phosphopantothenate 4'- Phosphopantothenoylcysteine H+ CO2 4'- phosphopantetheine ATP H+ PPi Dephospho-CoA ATP H+ ADP Coenzyme A ATP H+ Adenine 2'-(5- Triphosphoribosyl)- 3'-dephospho- CoA 5,10- Methylene-THF H2O Tetrahydrofolic acid 2- dehydropantoate NADPH H+ NADP (R)-pantoate Cytidine triphosphate L-Cysteine Cytidine monophosphate PPi H+ 2,3- Dihydroxyisovaleric acid H2O β-Alanine ATP AMP PPi H+ Valine Biosynthesis
Mitochondria Unknown Unknown Unknown CAB5 Unknown ECM31 PAN5 CAB2 ILV3 PAN6 α- Ketoisovaleric acid Pantothenic acid Adenosine triphosphate Adenosine diphosphate Hydrogen Ion D-4'- Phosphopantothenate 4'- Phosphopantothenoylcysteine Hydrogen Ion Carbon dioxide 4'- phosphopantetheine Adenosine triphosphate Hydrogen Ion Pyrophosphate Dephospho-CoA Adenosine triphosphate Hydrogen Ion Adenosine diphosphate Coenzyme A Adenosine triphosphate Hydrogen Ion Adenine 2'-(5- Triphosphoribosyl)- 3'-dephospho- CoA 5,10- Methylene-THF Water Tetrahydrofolic acid 2- dehydropantoate NADPH Hydrogen Ion NADP (R)-pantoate Cytidine triphosphate L-Cysteine Cytidine monophosphate Pyrophosphate Hydrogen Ion 2,3- Dihydroxyisovaleric acid Water β-Alanine Adenosine triphosphate Adenosine monophosphate Pyrophosphate Hydrogen Ion Valine Biosynthesis