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ENTRY	nt06019
Name	Steroid hormone biosynthesis
Category	Pathway view; Lipid/glycolipid metabolism
Pathway	hsa00140 Steroid hormone biosynthesis
Module	hsa_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
Disease	H00216 Congenital adrenal hyperplasia
Display	drug-target relation   disease type

D2  |     N00759    Cholesterol − CYP11A1 → Pregnenolone − HSD3B1/2 → Progesterone     Adrenal insufficiency     CYP11A1*     Adrenal hyperplasia             HSD3B2*                 D1  |             N00338    Progesterone − CYP17A1 ⇉ CYP21A2 ⇉ CYP11B1/2 → Cortisol − HSD11B2 → Cortisone     Adrenal hyperplasia     CYP17A1*     Adrenal hyperplasia         CYP21A2*     Adrenal hyperplasia             CYP11B1*     AME syndrome                     HSD11B2*   N00309            Cortisone+NADPH − HSD11B1 → Cortisol+NADP+     Cortisone reductase deficiency 2             HSD11B1* N00311    NADP+ − H6PD → NADPH     Cortisone reductase deficiency 1     H6PD* N00313                SERPINA6 − Cortisol     CBG deficiency             SERPINA6*                 D1  |     N00339    Progesterone − CYP21A2 ⇉ CYP11B1 ⇉ CYP11B2 → Aldosterone     Aldosterone synthase deficiency             CYP11B2*             D2  |                     N01541    Pregnenolone − CYP17A1 ⇉ HSD3B1/2 → Androstenedione − (AKR1C3,HSD17B) → T − SRD5A → DHT     46,XY DSD                 HSD17B3*     46,XY DSD                         SRD5A2*           D2  |                     N01802    Pregnenolone − CYP17A1 ⇉ HSD3B1/2 ⇉ SRD5A1 ⇉ (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 name	Disease category
Adrenal insufficiency	H02314	Adrenal insufficiency, congenital, with 46XY sex reversal, partial or complete	Endocrine and metabolic disease
Adrenal hyperplasia	H00216	Congenital adrenal hyperplasia	Endocrine and metabolic disease
AME syndrome	H00259	Apparent mineralocorticoid excess syndrome	Endocrine and metabolic disease
Cortisone reductase deficiency 2	H01111	Cortisone reductase deficiency	Endocrine and metabolic disease
Cortisone reductase deficiency 1	H01111	Cortisone reductase deficiency	Endocrine and metabolic disease
CBG deficiency	H01163	Corticosteroid-binding globulin (CBG) deficiency	Endocrine and metabolic disease
Aldosterone synthase deficiency	H00258	Aldosterone synthase deficiency	Endocrine and metabolic disease
46,XY DSD	H00608	46,XY disorder of sex development due to testosterone secretion defect	Reproductive system disease
46,XY GD	H00607	46,XY gonadal dysgenesis	Reproductive system disease
Aromatase deficiency	H02020	Aromatase deficiency	Endocrine and metabolic disease
Aromatase excess syndrome	H00794	Aromatase excess syndrome	Endocrine and metabolic disease

		Drug name
D1	D11062	Osilodrostat phosphate (JAN/USAN)
D2	D01180	Trilostane (JAN/USAN)