Increased activation of the alternative “backdoor” pathway in patients with 21-hydroxylase deficiency: evidence from urinary steroid hormone analysis
Abstract
BACKGROUND: 17-Hydroxyprogesterone (17-OHP) can be converted to dihydrotestosterone (DHT) via an alternative "backdoor" route that bypasses the conventional intermediates androstenedione and testosterone. In this backdoor pathway, 17-OHP is converted to 5α-pregnane-3α,17α-diol-20-one (pdiol), which is an excellent substrate for the 17,20 lyase activity of CYP17A1 to produce androsterone. OBJECTIVE AND HYPOTHESES: The objective of this study was to obtain evidence for the presence of the backdoor pathway in patients with 21-hydroxylase deficiency (21-OHD). METHODS: We compared urinary steroid hormone profiles determined by gas chromatography-mass spectrometry of 142 untreated 21-OHD patients (age range, 1 d to 25.4 yr; 51 males) with 138 control subjects. The activity of the backdoor pathway was assessed using the ratios of the urinary concentrations of pdiol to those of the metabolites of the classic Δ4 and Δ5 pathways. In contrast to etiocholanolone, which originates almost exclusively from the classic pathways, androsterone may be derived additionally from the backdoor pathway. Therefore, the androsterone to etiocholanolone ratio can be used as an indicator for the presence of the backdoor pathway. RESULTS: Untreated 21-OHD subjects showed increased urinary ratios of pdiol to the Δ4 and Δ5 pathway metabolites and a higher androsterone to etiocholanolone ratio. CONCLUSIONS: The elevated ratios of pdiol to the Δ4 and Δ5 pathway metabolites as well as the higher androsterone to etiocholanolone ratio in patients with 21-OHD indicate postnatal activity of the backdoor pathway with maximum activity during early infancy. Our data provide new insights into the pathophysiology of androgen biosynthesis of 21-OHD.
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Increased alternative pathway metabolite excretion has been described in patients with 21OHD deficiency.
“and 21OHD deficiency.126”
From Congenital adrenal hyperplasia (2017) · cites this paper as [126]
The role of the alternative pathway in congenital adrenal hyperplasia is evidenced by increased urinary levels of metabolites of its unique steroidal intermediates in infants, children, and adults with the condition.
“The role of the alternative pathway in CAH is evidenced by increased levels of metabolites of its unique steroidal intermediates in the urine of infants, children, and adults with CAH (26).”
From Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline (2018) · cites this paper as [26]
Evidence for the alternative pathway in CAH comes from higher urinary levels of metabolites of its unique steroid intermediates in affected infants, children, and adults.
“The role of the alternative pathway in CAH is evidenced by increased levels of metabolites of its unique steroidal intermediates in the urine of infants, children, and adults with CAH(26).”
From Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline (2018) · cites this paper as [26]
Virilization in utero in individuals with 21-hydroxylase deficiency is likely due to excessive backdoor androgen synthesis that is occurring despite the absence of testes.
“It is likely that virilization in utero in individuals with 21-hydroxylase deficiency is due to excessive backdoor androgen synthesis that is occurring despite the absence of testes [51]”
From Alternative (backdoor) androgen production and masculinization in the human fetus (2019) · cites this paper as [51]
Androsterone can be reactivated by sequential 17β-reduction and 3α-oxidase reactions.
“Androsterone, considered an inactive metabolite of DHT under normal circumstances, can then be reactivated by the sequential 17β-reduction and 3α-oxidase reactions [[63], [64], [65]].”
From Human steroid biosynthesis, metabolism and excretion are differentially reflected by serum and urine steroid metabolomes: a comprehensive review (2019) · cites this paper as [63]
In humans, the backdoor pathway is relevant in pathological conditions when 17OHPROG accumulates, such as in congenital adrenal hyperplasia due to PORD or 21-hydroxylase deficiency.
“In humans, the backdoor pathway is relevant in pathological conditions when 17OHPROG accumulates, such as in congenital adrenal hyperplasia due to cytochrome P450 oxidoreductase deficiency (PORD) or 21-hydroxylase deficiency (Arlt et al., 2004, Homma et al., 2006, Kamrath et al., 2012, Krone et al., 2012, Miller and Auchus, 2011).”
From Intracrine androgen biosynthesis, metabolism and action revisited (2018) · cites this paper as Kamrath et al., 2012
Alternative DHT pathway activity is reflected by 5α17HP and An.
“Alternative DHT pathway activity is reflected by 5α17HP and An (22, 26)”
From Steroid Metabolome Analysis in Disorders of Adrenal Steroid Biosynthesis and Metabolism (2019) · cites this paper as [26]
Increased 11-oxygenated pathway activity is reflected by the major metabolite of 11OHA4, 11β-OHAn.
“whereas increased 11-oxygenated pathway activity is reflected by the major metabolite of 11OHA4, 11β-OHAn (22, 25, 26)”
From Steroid Metabolome Analysis in Disorders of Adrenal Steroid Biosynthesis and Metabolism (2019) · cites this paper as [26]
Accumulating 17OHP also drives increased androgen production by the alternative DHT pathway.
“Accumulating 17OHP also drives increased androgen production by the alternative DHT pathway (26)”
From Steroid Metabolome Analysis in Disorders of Adrenal Steroid Biosynthesis and Metabolism (2019) · cites this paper as [26]
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“It has been speculated that the backdoor pathway could be involved in androgen synthesis in patients with 21-OHD (4, 10, 11); however, no data have yet been published supporting this hypothesis.”
BackgroundCongenital adrenal hyperplasia due to 21-hydroxylase deficiency*
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- Authors
- Kamrath, C., Hochberg, Z., Hartmann, M. F., Remer, T., & Wudy, S. A.
- Affiliations
- Justus-Liebig-Universität Gießen (DE)
- Technion – Israel Institute of Technology (IL)
- Forschungsinstitut für Kinderernährung (DE)
- Corresponding author
- Kamrath, Clemens
- Venue
- The Journal of Clinical Endocrinology & Metabolism
- Publisher
- The Endocrine Society
- Published
- 2012-03-01
- Volume
- 97
- Issue
- 3
- Pages
- E367-E375
- ISSN
- 0021-972X (ISSN-L)
- 1945-7197
- OpenAlex
- W2132144622
- Cited by
- 191
- References
- 31
- Citations by year
- 2026: 6 · 2025: 8 · 2024: 8 · 2023: 10 · 2022: 11 · 2021: 16 · 2020: 11 · 2019: 20 · 2018: 15 · 2017: 22 · 2016: 16 · 2015: 11 · 2014: 15 · 2013: 11 · 2012: 8
- FWCI
- 4.53
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Steroid Chemistry and Biochemistry
- Keywords
- 21-hydroxylase deficiency, congenital adrenal hyperplasia, 17-hydroxyprogesterone, androsterone, etiocholanolone, CYP17A1, 17,20-lyase, androgen production, urinary steroids
- MeSH terms
- Adolescent, Adrenal Hyperplasia, Congenital, Adult, Androstenedione, Androsterone, Child, Child, Preschool, Etiocholanolone, Female, Humans, Infant, Infant, Newborn, Male, Gonadal Steroid Hormones, Dihydrotestosterone, Steroid 17-alpha-Hydroxylase
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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