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 [https://www.wikipathways.org/index.php/Pathway:WP4524].
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 [http://www.annualreviews.org/doi/abs/10.1146/annurev.med.48.1.231].
b0e
e3e
e11
also known as CYP11A1
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e11
e3e
Reference study in mice [PMID:9405715]
b6c
This enzyme produces NADPH which is neccessary for 11beta-HSD1 to convert cortisone into cortisol.
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e3e
e11
e11
e3e
e11
e3e
EC number: 1.14.15.5
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e3e
SPs:DIDH RAT
1.1.1.53, similiar SP:2BHD STREX
aka 4,5beta-Dihydrocortisone
1.3.1.3
SPs:DIDH RAT
1.1.1.53, similiar SP:2BHD STREX
AKA Tetrahydrocortisol
AKA 11-BETA,17-ALPHA,21-TRIHYDROXY-5-BETA-PREGNANE-3,20-DIONE
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f5d
cc0
d0a
e35
a2f
e30
cc0
ff1
Reference study in mice [PMID:9405715]
b6c
f15
e3e
e11
e3e
e11
congenital adrenal hyperplasia
DOID:0050811
Disease
8001864
PubMed
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PW:0001478
Pathway Ontology
8839934
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DOID:4367
Disease
C19-steroid hormone biosynthetic pathway
PW:0000770
Pathway Ontology
12788846
PubMed
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Disease
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Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Disease'
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Dionisi-Vici
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PW:0001152
Pathway Ontology
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PW:0000772
Pathway Ontology
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PW:0001479
Pathway Ontology
congenital adrenal insufficiency
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Disease
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