KEGG    Network variation - Steroid hormone biosynthesis
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ENTRYnt06019
NameSteroid hormone biosynthesis
CategoryPathway view; Lipid/glycolipid metabolism
Pathwayhsa00140 Steroid hormone biosynthesis
Modulehsa_M00107 Steroid hormone biosynthesis, cholesterol => pregnenolone => progesterone
hsa_M00108 C21-Steroid hormone biosynthesis, progesterone => corticosterone/aldosterone
hsa_M00109 C21-Steroid hormone biosynthesis, progesterone => cortisol/cortisone
hsa_M00110 C19/C18-Steroid hormone biosynthesis, pregnenolone => androstenedione => estrone
hsa_M00976 C19-Steroid hormone biosynthesis, pregnenolone => testosterone => dihydrotestosterone
hsa_M00977 C19-Steroid hormone biosynthesis (androgen backdoor pathway), pregnenolone => androsterone => dihydrotestosterone
DiseaseH00216 Congenital adrenal hyperplasia
Display drug-target relation   disease type
       D2
 |
  
N00759    CholesterolCYP11A1PregnenoloneHSD3B1/2Progesterone
    Adrenal insufficiency   CYP11A1*
    Adrenal hyperplasia       HSD3B2*
       D1
 |
      
N00338    ProgesteroneCYP17A1CYP21A2CYP11B1/2CortisolHSD11B2Cortisone
    Adrenal hyperplasia   CYP17A1*
    Adrenal hyperplasia     CYP21A2*
    Adrenal hyperplasia       CYP11B1*
    AME syndrome           HSD11B2*
N00309        Cortisone+NADPHHSD11B1Cortisol+NADP+
    Cortisone reductase deficiency 2       HSD11B1*
N00311    NADP+H6PDNADPH
    Cortisone reductase deficiency 1   H6PD*
N00313          SERPINA6Cortisol
    CBG deficiency       SERPINA6*
       D1
 |
  
N00339    ProgesteroneCYP21A2CYP11B1CYP11B2Aldosterone
    Aldosterone synthase deficiency       CYP11B2*
     D2
 |
          
N01541    PregnenoloneCYP17A1HSD3B1/2Androstenedione(AKR1C3,HSD17B)TSRD5ADHT
    46,XY DSD         HSD17B3*
    46,XY DSD             SRD5A2*
     D2
 |
          
N01802    PregnenoloneCYP17A1HSD3B1/2SRD5A1(HSD17B6,AKR1C2/4)(AKR1C3,HSD17B3)(AKR1C2,HSD17B6)DHT
    46,XY GD         AKR1C2*
    46,XY GD         AKR1C4*
N01540    [Androstenedione,T..CYP19A1[E1,E2]
    Aromatase deficiency   CYP19A1*
    Aromatase excess syndrome   CYP19A1*

Disease nameDisease category
Adrenal insufficiencyH02314Adrenal insufficiency, congenital, with 46XY sex reversal, partial or completeEndocrine and metabolic disease
Adrenal hyperplasiaH00216Congenital adrenal hyperplasiaEndocrine and metabolic disease
AME syndromeH00259Apparent mineralocorticoid excess syndromeEndocrine and metabolic disease
Cortisone reductase deficiency 2H01111Cortisone reductase deficiencyEndocrine and metabolic disease
Cortisone reductase deficiency 1H01111Cortisone reductase deficiencyEndocrine and metabolic disease
CBG deficiencyH01163Corticosteroid-binding globulin (CBG) deficiencyEndocrine and metabolic disease
Aldosterone synthase deficiencyH00258Aldosterone synthase deficiencyEndocrine and metabolic disease
46,XY DSDH0060846,XY disorder of sex development due to testosterone secretion defectReproductive system disease
46,XY GDH0060746,XY gonadal dysgenesisReproductive system disease
Aromatase deficiencyH02020Aromatase deficiencyEndocrine and metabolic disease
Aromatase excess syndromeH00794Aromatase excess syndromeEndocrine and metabolic disease


Drug name
D1D11062Osilodrostat phosphate (JAN/USAN)
D2D01180Trilostane (JAN/USAN)