Carnitine Palmitoyl Transferase Deficiency II

Metabolites

(S)-3-Hydroxydodecanoyl-CoA

Formula: C33H58N7O18P3S (965.2771758)

CAS ID: 72059-49-5

(S)-3-Hydroxyhexadecanoyl-CoA

Formula: C37H66N7O18P3S (1021.3397726)

CAS ID: 35106-50-4

(S)-3-Hydroxyoctanoyl-CoA

Formula: C29H50N7O18P3S (909.2145790000001)

CAS ID: 79171-48-5

(S)-3-Hydroxytetradecanoyl-CoA

Formula: C35H62N7O18P3S (993.3084742)

CAS ID:

(S)-Hydroxydecanoyl-CoA

Formula: C31H54N7O18P3S (937.2458774)

CAS ID: 6245-70-1

(S)-Hydroxyhexanoyl-CoA

Formula: C27H46N7O18P3S (881.1832806000001)

CAS ID: 79171-47-4

3-Oxodecanoyl-CoA

Formula: C31H52N7O18P3S (935.2302281999999)

CAS ID: 50411-91-1

3-Oxododecanoyl-CoA

Formula: C33H56N7O18P3S (963.2615266000001)

CAS ID: 78303-19-2

3-Oxohexanoyl-CoA

Formula: C27H44N7O18P3S (879.1676314)

CAS ID: 19774-86-8

3-Oxooctanoyl-CoA

Formula: C29H48N7O18P3S (907.1989298)

CAS ID: 54684-64-9

3-Oxopalmitoyl-CoA

Formula: C37H64N7O18P3S (1019.3241234000001)

CAS ID: 34619-89-1

3-Oxotetradecanoyl-CoA

Formula: C35H60N7O18P3S (991.2928250000001)

CAS ID: 122364-86-7

Acetyl-CoA

Formula: C23H38N7O17P3S (809.1257688000001)

CAS ID: 72-89-9

AMP

Formula: C10H14N5O7P (347.06308240000004)

CAS ID: 61-19-8

ATP

Formula: C10H16N5O13P3 (506.9957476)

CAS ID: 56-65-5

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

Decanoyl-CoA

Formula: C31H54N7O17P3S (921.2509624)

CAS ID: 1264-57-9

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

Hexadecanoic acid

Formula: C16H32O2 (256.2402172)

CAS ID: 57-10-3

Hexanoyl-CoA

Formula: C27H46N7O17P3S (865.1883656000001)

CAS ID: 5060-32-2

L-Carnitine

Formula: C7H15NO3 (161.105188)

CAS ID: 541-15-1

L-Palmitoylcarnitine

Formula: C23H45NO4 (399.33484100000004)

CAS ID: 2364-67-2

Lauroyl-CoA

Formula: C33H58N7O17P3S (949.2822608)

CAS ID: 6244-92-4

NAD+

Formula: C21H28N7O14P2 (664.1169428000001)

CAS ID: 53-84-9

NADH

Formula: C21H29N7O14P2 (665.1247674)

CAS ID: 58-68-4

Octanoyl-CoA

Formula: C29H50N7O17P3S (893.2196640000001)

CAS ID: 1264-52-4

Tetradecanoyl-CoA

Formula: C35H62N7O17P3S (977.3135592)

CAS ID: 3130-72-1

trans-Dec-2-enoyl-CoA

Formula: C31H52N7O17P3S (919.2353132)

CAS ID: 10018-95-8

trans-Hex-2-enoyl-CoA

Formula: C27H44N7O17P3S (863.1727164)

CAS ID: 10018-93-6

trans-Oct-2-enoyl-CoA

Formula: C29H48N7O17P3S (891.2040148)

CAS ID: 10018-94-7

trans-Tetradec-2-enoyl-CoA

Formula: C35H60N7O17P3S (975.2979100000001)

CAS ID: 38795-33-4

Butyryl-CoA

Formula: C25H42N7O17P3S (837.1570672000001)

CAS ID: 2140-48-9

diphosphate

Formula: O7P2 (173.911931)

CAS ID: 14000-31-8



Enzyme

EC Number name full name note
2.3.1.-
1.1.1.211 long-chain-3-hydroxyacyl-CoA dehydrogenase long-chain-(S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase
1.3.8.1 short-chain acyl-CoA dehydrogenase short-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
1.3.8.5 short-chain 2-methylacyl-CoA dehydrogenase short-chain 2-methylacyl-CoA:electron-transfer flavoprotein 2-oxidoreductase
1.3.8.6 glutaryl-CoA dehydrogenase (ETF) glutaryl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase (decarboxylating)
1.3.8.7 medium-chain acyl-CoA dehydrogenase medium-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
1.3.8.8 long-chain acyl-CoA dehydrogenase long-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
1.3.8.9 very-long-chain acyl-CoA dehydrogenase very-long-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
2.3.1.21 carnitine O-palmitoyltransferase palmitoyl-CoA:L-carnitine O-palmitoyltransferase
4.2.1.17 enoyl-CoA hydratase (3S)-3-hydroxyacyl-CoA hydro-lyase
5.3.3.8 DELTA3-DELTA2-enoyl-CoA isomerase (3Z/3E)-alk-3-enoyl-CoA (2E)-isomerase
6.2.1.15 arachidonate-CoA ligase arachidonate:CoA ligase (AMP-forming)
6.2.1.24 phytanate-CoA ligase phytanate:CoA ligase (AMP-forming)
6.2.1.3 long-chain-fatty-acid-CoA ligase long-chain fatty acid:CoA ligase (AMP-forming)


Proteins

Protein ID name full name