Classical pathway of steroidogenesis with glucocorticoid and mineralocorticoid metabolism (WP4523)

The biosynthesis of steroid hormones is a difficult process in which Cholesterol is transformed into mineralocorticoids, glucocorticoids and sex hormones via a series of hydroxylation, oxidation and reduction steps. To better understand the molecular level of sexual organ maturation in humans, the classical pathway and the alternative pathway of this process are produced. The pathways produce the main steroid hormones in humans, namely Progestogen, Corticosteroids, Androgens and Estrogens. The classical pathway is meant to produce an important steroid called Androgen, which is a synthetic steroid hormone that regulates sexual development and the maintenance of the male sex organs via binding to androgen receptors. Next to the classical pathway of androgen synthesis, alternative pathways are known, such as []. For more information and details about Androgens and the diseases linked with this molecular pathway, please visit Chapter 37 of the book of Blau (ISBN 3642403360 (978-3642403361)) . We have recently expanded this pathway with information from the Glucocorticoid and Mineralocorticoid Metabolism (previously captured in WP273; overlapping content is indicated with double borders for individual datanodes; information previously missing is added with dashed borders). Mineralocorticoid (M) and glucocorticoid (G) receptors regulate transcription; either through 11-beta-hydroxysteroid dehydrogenase influencing aldosterone specificity on epithelial M-receptors or by modulcation of AP-1- and NF-kappa-B-induced transcription through G-receptors. Specifically for the first case, aldosterone resistance in an autosomal form (aka pseudohypoaldosteronism) is linked to loss-of-function in epithelical Na-channel subunits [].
last edited


ElineSanders, Ingebude, DeSl, IreneHemel, Egonw, Fehrhart, Eweitz, and Finterly

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Homo sapiens


Inborn Errors of Metabolism (IEM) Pathways Rare Diseases


Pathway Ontology: steroid biosynthetic pathway congenital adrenal hyperplasia pathway C19-steroid hormone biosynthetic pathway apparent mineralocorticoid excess syndrome pathway lipoid congenital adrenal hyperplasia pathway C21-steroid hormone biosynthetic pathway glucocorticoid biosynthetic pathway disease pathway

Disease Ontology: pseudohermaphroditism obsolete apparent mineralocorticoid excess congenital adrenal insufficiency congenital adrenal hyperplasia cortisone reductase deficiency apparent mineralocorticoid excess syndrome


Label Type Compact Identifier
Corticosterone Methyl Oxidase Protein ensembl:ENSG00000179142
P450scc Protein ensembl:ENSG00000140459
Dihydrotestosterone Metabolite chebi:16330
P450c21 Protein ensembl:ENSG00000231852
Testosterone Metabolite chebi:17347
Oestradiol Metabolite chebi:16469
11beta-HSD1 Protein ensembl:ENSG00000117594
Cholesterol Metabolite chebi:16113
17-hydroxyprogesterone Metabolite chebi:17252
Cortisone Metabolite chebi:16962
3-beta-HSD Protein ensembl:ENSG00000203859
Progesterone Metabolite chebi:17026
STAR Protein ensembl:ENSG00000147465
Corticosterone Metabolite chebi:16827
11-Deoxycortisol Metabolite chebi:28324
DHEA Metabolite chebi:28689
Cortisol Metabolite wikidata:Q190875
11beta-HSD2 Protein ensembl:ENSG00000176387
Androstenedione Metabolite chebi:16422
(11)-Deoxycorticosterone Metabolite chebi:16973
POR Protein uniprot:P16435
POR Protein uniprot:P16435
17-beta-HSD3 Protein ensembl:ENSG00000130948
H6PD Protein ensembl:ENSG00000049239
18-hydroxycorticosterone Metabolite chebi:16485
POR Protein uniprot:P16435
Cytochrome b5 Protein uniprot:J3KNC7
P450c17 Protein ensembl:ENSG00000148795
Aldosterone Metabolite chebi:27584
17-hydroxypregnenolone Metabolite chebi:28750
5-alpha-Reductase2 Protein ensembl:ENSG00000277893
P450Aro Protein ensembl:ENSG00000137869
Pregnenolone Metabolite chebi:16581
3-beta-HSD Protein ensembl:ENSG00000203859
P450c11 Protein ensembl:ENSG00000160882
P450c17 Protein ensembl:ENSG00000148795
P450c17 Protein ensembl:ENSG00000148795
3-beta-HSD Protein ensembl:ENSG00000203859
POR Protein uniprot:P16435
NADP+ Metabolite chebi:58349
NADPH Metabolite chebi:57783
Cholesterol Biosynthesis Pathway wikipathways:WP197
CYP11A1 GeneProduct ncbigene:1583
HSD3B1 Protein ncbigene:3283
3B-OH-delta-Steroid Dh Protein eccode:
HSD3B1 GeneProduct ncbigene:3283
HSD3B2 GeneProduct ncbigene:3284
Androgen Synthesis and Metabolism Pathway wikipathways:WP496
CYP11B2 GeneProduct ncbigene:1585
Corticosterone 18-Monooxy Protein eccode:
11b, 21-Dihydroxy-3,20-5b-Pregnan-18-al Metabolite pubchem.compound:44263338
3a,11b,21-Trihydroxy-20-Oxo-5b-Pregnan-18-al Metabolite pubchem.compound:44263346
3-Oxo-5b-Steroid Dh Protein uniprot:3O5B
3a-Hydroxy-steroid Dh Protein eccode:
3a-OH-5b-Pregnane-20-one Metabolite pubchem.compound:24779614
5b-Pregnane-3,20-dione Metabolite pubchem.compound:92745
20b-Hydroxy-steroid Dh Protein eccode:
Pregnanediol Metabolite cas:80-92-2
Glucuronides Metabolite chebi:26763
17a,21-Dihydroxy-5b-17a,21-Dihydroxy-5b-Pregnane-3,11,20-trione Metabolite pubchem.compound:65554
Urocortisone Metabolite pubchem.compound:5754
Cortolone Metabolite cas:516-42-7
Cortisone beta-reductase Protein eccode:
3a-Hydroxy-steroid Dh Protein eccode:
(R)20-hydroxy-steroid Dh Protein eccode:
Urocortisol Metabolite pubchem.compound:5864
11b,17a 21-Trihydroxy-5bPregnane 3,20-dione Metabolite cas:1482-50-4
Cortisol Metabolite wikidata:Q190875
Steroid Hormone Signaling Pathway wikipathways:WP170


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