KEGG   PATHWAY: hsa00071Help
Entry
hsa00071                    Pathway                                

Name
Fatty acid degradation - Homo sapiens (human)
Class
Metabolism; Lipid metabolism
BRITE hierarchy
Pathway map
Fatty acid degradation
hsa00071

All organismsOrtholog table
Module
beta-Oxidation, acyl-CoA synthesis [PATH:hsa00071]
beta-Oxidation [PATH:hsa00071]
Disease
H00162  
Sjogren-Larsson syndrome
H00178  
Glutaric acidemia
H00407  
Peroxisomal beta-oxidation enzyme deficiency
H00525  
Disorders of fatty-acid oxidation
H01267  
Familial hyperinsulinemic hypoglycemia (HHF)
H01352  
Mitochondrial trifunctional protein (TFP) deficiency
H01364  
3-Hydroxyacyl-CoA dehydrogenase deficiency
H01400  
Secondary hyperammonemia
Drug
D00123  
Cyanamide (JP16)
D00131  
Disulfiram (JP16/USP/INN)
D00707  
Fomepizole (JAN/USAN/INN)
D05292  
Oxfenicine (USAN/INN)
Organism
Homo sapiens (human) [GN:hsa]
Gene
39  
ACAT2; acetyl-CoA acetyltransferase 2 [KO:K00626] [EC:2.3.1.9]
38  
ACAT1; acetyl-CoA acetyltransferase 1 [KO:K00626] [EC:2.3.1.9]
30  
ACAA1; acetyl-CoA acyltransferase 1 [KO:K07513] [EC:2.3.1.16]
10449  
ACAA2; acetyl-CoA acyltransferase 2 [KO:K07508] [EC:2.3.1.16]
3032  
HADHB; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit [KO:K07509] [EC:2.3.1.16]
3033  
HADH; hydroxyacyl-CoA dehydrogenase [KO:K00022] [EC:1.1.1.35]
3030  
HADHA; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [KO:K07515] [EC:1.1.1.211 4.2.1.17]
1962  
EHHADH; enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase [KO:K07514] [EC:5.3.3.8 1.1.1.35 4.2.1.17]
1892  
ECHS1; enoyl CoA hydratase, short chain, 1, mitochondrial [KO:K07511] [EC:4.2.1.17]
8310  
ACOX3; acyl-CoA oxidase 3, pristanoyl [KO:K00232] [EC:1.3.3.6]
51  
ACOX1; acyl-CoA oxidase 1, palmitoyl [KO:K00232] [EC:1.3.3.6]
35  
ACADS; acyl-CoA dehydrogenase, C-2 to C-3 short chain [KO:K00248] [EC:1.3.8.1]
34  
ACADM; acyl-CoA dehydrogenase, C-4 to C-12 straight chain [KO:K00249] [EC:1.3.8.7]
33  
ACADL; acyl-CoA dehydrogenase, long chain [KO:K00255] [EC:1.3.8.8]
36  
ACADSB; acyl-CoA dehydrogenase, short/branched chain [KO:K09478] [EC:1.3.99.12]
37  
ACADVL; acyl-CoA dehydrogenase, very long chain [KO:K09479] [EC:1.3.8.9]
2639  
GCDH; glutaryl-CoA dehydrogenase [KO:K00252] [EC:1.3.8.6]
23305  
ACSL6; acyl-CoA synthetase long-chain family member 6 [KO:K01897] [EC:6.2.1.3]
2182  
ACSL4; acyl-CoA synthetase long-chain family member 4 [KO:K01897] [EC:6.2.1.3]
2180  
ACSL1; acyl-CoA synthetase long-chain family member 1 [KO:K01897] [EC:6.2.1.3]
51703  
ACSL5; acyl-CoA synthetase long-chain family member 5 [KO:K01897] [EC:6.2.1.3]
2181  
ACSL3; acyl-CoA synthetase long-chain family member 3 [KO:K01897] [EC:6.2.1.3]
23205  
ACSBG1; acyl-CoA synthetase bubblegum family member 1 [KO:K15013] [EC:6.2.1.3]
81616  
ACSBG2; acyl-CoA synthetase bubblegum family member 2 [KO:K15013] [EC:6.2.1.3]
1374  
CPT1A; carnitine palmitoyltransferase 1A (liver) [KO:K08765] [EC:2.3.1.21]
1375  
CPT1B; carnitine palmitoyltransferase 1B (muscle) [KO:K08765] [EC:2.3.1.21]
126129  
CPT1C; carnitine palmitoyltransferase 1C [KO:K08765] [EC:2.3.1.21]
1376  
CPT2; carnitine palmitoyltransferase 2 [KO:K08766] [EC:2.3.1.21]
1632  
ECI1; enoyl-CoA delta isomerase 1 [KO:K13238] [EC:5.3.3.8]
10455  
ECI2; enoyl-CoA delta isomerase 2 [KO:K13239] [EC:5.3.3.8]
1579  
CYP4A11; cytochrome P450, family 4, subfamily A, polypeptide 11 [KO:K17687] [EC:1.14.15.3]
284541  
CYP4A22; cytochrome P450, family 4, subfamily A, polypeptide 22 [KO:K17688] [EC:1.14.15.3]
124  
ADH1A; alcohol dehydrogenase 1A (class I), alpha polypeptide [KO:K13951] [EC:1.1.1.1]
125  
ADH1B; alcohol dehydrogenase 1B (class I), beta polypeptide [KO:K13951] [EC:1.1.1.1]
126  
ADH1C; alcohol dehydrogenase 1C (class I), gamma polypeptide [KO:K13951] [EC:1.1.1.1]
131  
ADH7; alcohol dehydrogenase 7 (class IV), mu or sigma polypeptide [KO:K13951] [EC:1.1.1.1]
127  
ADH4; alcohol dehydrogenase 4 (class II), pi polypeptide [KO:K13980] [EC:1.1.1.1]
128  
ADH5; alcohol dehydrogenase 5 (class III), chi polypeptide [KO:K00121] [EC:1.1.1.1 1.1.1.284]
130  
ADH6; alcohol dehydrogenase 6 (class V) [KO:K13952] [EC:1.1.1.1]
217  
ALDH2; aldehyde dehydrogenase 2 family (mitochondrial) [KO:K00128] [EC:1.2.1.3]
224  
ALDH3A2; aldehyde dehydrogenase 3 family, member A2 [KO:K00128] [EC:1.2.1.3]
219  
ALDH1B1; aldehyde dehydrogenase 1 family, member B1 [KO:K00128] [EC:1.2.1.3]
501  
ALDH7A1; aldehyde dehydrogenase 7 family, member A1 [KO:K14085] [EC:1.2.1.3 1.2.1.8 1.2.1.31]
223  
ALDH9A1; aldehyde dehydrogenase 9 family, member A1 [KO:K00149] [EC:1.2.1.3 1.2.1.47]
Compound
C00010  
CoA
C00024  
Acetyl-CoA
C00071  
Aldehyde
C00136  
Butanoyl-CoA
C00154  
Palmitoyl-CoA
C00162  
Fatty acid
C00226  
Primary alcohol
C00229  
Acyl-carrier protein
C00249  
Hexadecanoic acid
C00332  
Acetoacetyl-CoA
C00340  
Reduced rubredoxin
C00435  
Oxidized rubredoxin
C00489  
Glutarate
C00517  
Hexadecanal
C00527  
Glutaryl-CoA
C00638  
Long-chain fatty acid
C00823  
1-Hexadecanol
C00877  
Crotonoyl-CoA
C01144  
(S)-3-Hydroxybutanoyl-CoA
C01371  
Alkane
C01832  
Lauroyl-CoA
C01944  
Octanoyl-CoA
C02593  
Tetradecanoyl-CoA
C02990  
L-Palmitoylcarnitine
C03221  
2-trans-Dodecenoyl-CoA
C03547  
omega-Hydroxy fatty acid
C03561  
(R)-3-Hydroxybutanoyl-CoA
C05102  
alpha-Hydroxy fatty acid
C05258  
(S)-3-Hydroxyhexadecanoyl-CoA
C05259  
3-Oxopalmitoyl-CoA
C05260  
(S)-3-Hydroxytetradecanoyl-CoA
C05261  
3-Oxotetradecanoyl-CoA
C05262  
(S)-3-Hydroxydodecanoyl-CoA
C05263  
3-Oxododecanoyl-CoA
C05264  
(S)-Hydroxydecanoyl-CoA
C05265  
3-Oxodecanoyl-CoA
C05266  
(S)-3-Hydroxyoctanoyl-CoA
C05267  
3-Oxooctanoyl-CoA
C05268  
(S)-Hydroxyhexanoyl-CoA
C05269  
3-Oxohexanoyl-CoA
C05270  
Hexanoyl-CoA
C05271  
trans-Hex-2-enoyl-CoA
C05272  
trans-Hexadec-2-enoyl-CoA
C05273  
trans-Tetradec-2-enoyl-CoA
C05274  
Decanoyl-CoA
C05275  
trans-Dec-2-enoyl-CoA
C05276  
trans-Oct-2-enoyl-CoA
C05279  
trans,cis-Lauro-2,6-dienoyl-CoA
C05280  
cis,cis-3,6-Dodecadienoyl-CoA
C20683  
Long-chain acyl-[acyl-carrier protein]
Reference
PMID:869535
  Authors
Parekh VR, Traxler RW, Sobek JM
  Title
N-Alkane oxidation enzymes of a pseudomonad.
  Journal
Appl Environ Microbiol 33:881-4 (1977)
KO pathway
 

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