KEGG   PATHWAY: hsa00071Help
Entry
hsa00071                    Pathway                                

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

Ortholog table
Module
hsa_M00086  beta-Oxidation, acyl-CoA synthesis [PATH:hsa00071]
hsa_M00087  beta-Oxidation [PATH:hsa00071]
Disease
H00162  Sjogren-Larsson syndrome
H00178  Glutaric acidemia
H00375  SBCAD deficiency
H00392  VLCAD deficiency
H00488  MCAD deficiency
H00525  Disorders of mitochondrial fatty-acid oxidation
H01267  Familial hyperinsulinemic hypoglycemia (HHF)
H01352  Mitochondrial trifunctional protein deficiency
H01364  3-Hydroxyacyl-CoA dehydrogenase deficiency
H01400  Secondary hyperammonemia
H01980  SCAD deficiency
H01981  Carnitine palmitoyltransferase I deficiency
H01982  Carnitine palmitoyltransferase II deficiency
H02096  Peroxisomal acyl-CoA oxidase deficiency
Drug
D00123  Cyanamide (JP17)
D00131  Disulfiram (JP17/USP/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 trifunctional multienzyme complex subunit beta [KO:K07509] [EC:2.3.1.16]
3033  HADH; hydroxyacyl-CoA dehydrogenase [KO:K00022] [EC:1.1.1.35]
3030  HADHA; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha [KO:K07515] [EC:1.1.1.211 4.2.1.17]
1962  EHHADH; enoyl-CoA hydratase and 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 [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 [KO:K00232] [EC:1.3.3.6]
35  ACADS; acyl-CoA dehydrogenase short chain [KO:K00248] [EC:1.3.8.1]
34  ACADM; acyl-CoA dehydrogenase medium 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 [KO:K08765] [EC:2.3.1.21]
1375  CPT1B; carnitine palmitoyltransferase 1B [KO:K19523] [EC:2.3.1.21]
126129  CPT1C; carnitine palmitoyltransferase 1C [KO:K19524] [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 member 11 [KO:K17687] [EC:1.14.15.3]
284541  CYP4A22; cytochrome P450 family 4 subfamily A member 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 member [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
ko00071   

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