KEGG    Network variation - beta-Oxidation in mitochondria
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ENTRYnt06020
Namebeta-Oxidation in mitochondria
CategoryPathway view; Lipid/glycolipid metabolism
Pathwayhsa00071 Fatty acid degradation
Modulehsa_M00086 beta-Oxidation, acyl-CoA synthesis
hsa_M00087 beta-Oxidation
DiseaseH00525 Disorders of mitochondrial fatty-acid oxidation
Display drug-target relation   disease type
N00812    Carnitine(blood)SLC22A5Carnitine
    CDSP   SLC22A5*
N00814    Palmitoylcarnitine..SLC25A20Palmitoylcarnitine..
    CACT deficiency   SLC25A20*
N00765    Palmitate(ACSL,ACSBG)CPT1A/B/CCPT2Palmitoyl-CoA
    CPT I deficiency     CPT1A*
    CPT II deficiency       CPT2*
N00804    Acyl-CoA(n)(ACADM,ACADL,ACADV..((ECHS1+HADH),HADH..(ACAA2,HADHB,ACAT1..Acyl-CoA(n-2)
    MCAD deficiency   ACADM*
    VLCAD deficiency   ACADVL*
    SCAD deficiency   ACADS*
    ECHS1 deficiency     ECHS1*
    HADH deficiency     HADH*
    LCHAD deficiency     HADHA*
    LCKAT deficiency       HADHB*

Disease nameDisease category
CDSPH01589Systemic primary carnitine deficiencyInherited metabolic disorder
CACT deficiencyH01983Carnitine-acylcarnitine translocase deficiencyInherited metabolic disorder
CPT I deficiencyH01981Carnitine palmitoyltransferase I deficiencyInherited metabolic disorder, Mitochondrial disease
CPT II deficiencyH01982Carnitine palmitoyltransferase II deficiencyInherited metabolic disorder, Mitochondrial disease
MCAD deficiencyH00488MCAD deficiencyInherited metabolic disorder, Mitochondrial disease
VLCAD deficiencyH00392VLCAD deficiencyInherited metabolic disorder
SCAD deficiencyH01980SCAD deficiencyInherited metabolic disorder, Mitochondrial disease
ECHS1 deficiencyH00525Disorders of mitochondrial fatty-acid oxidationInherited metabolic disorder
HADH deficiencyH013643-Hydroxyacyl-CoA dehydrogenase deficiencyInherited metabolic disorder, Mitochondrial disease
LCHAD deficiencyH00489LCHAD deficiencyInherited metabolic disorder
LCKAT deficiencyH01352Mitochondrial trifunctional protein deficiencyInherited metabolic disorder, Mitochondrial disease