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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
+
CO
2
4'-
phosphopantetheine
ATP
H
+
PP
i
Dephospho-CoA
ATP
H
+
ADP
Coenzyme A
ATP
H
+
Adenine
2'-(5-
Triphosphoribosyl)-
3'-dephospho-
CoA
5,10-
Methylene-THF
H
2
O
Tetrahydrofolic
acid
2-
dehydropantoate
NADPH
H
+
NADP
(R)-pantoate
Cytidine
triphosphate
L-Cysteine
Cytidine
monophosphate
PP
i
H
+
2,3-
Dihydroxyisovaleric
acid
H
2
O
β-Alanine
ATP
AMP
PP
i
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