11beta-Hydroxyprogesterone

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11beta-Hydroxyprogesterone
2D structure for 11beta-Hydroxyprogesterone
Chemical Name	17-acetyl-11-hydroxy-10,13-dimethyl-1,2,6,7,8,9,11,12,14,15,16,17-dodecahydrocyclopenta[a]phenanthren-3-one
Chemical Formula	C₂₀H₂₈O₄
CAS Number	600-57-7
Chemical Information	HMDB04031
Biochemical Taxonomy	Steroids and Steroid Derivatives
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Not Available
Biofluid Location	Blood
Tissue Location	Adrenal Cortex Adrenal Gland Bladder Epidermis Fibroblasts Gonads Intestine Kidney Muscle Myelin Nerves Neurons Placenta Placental Platelet Prostate Skeletal Muscle Spleen Testes Testis Thyroid Gland Adipose Tissue
Normal Biofluid Concentrations	Blood: 0.00002 +/- 0.00001 uM
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Not Available
Other (Monogenic Disorders)	Not Available
Abnormal Biofluid Concentrations	Not Available
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
11beta-Hydroxyprogesterone is a normal human metabolite. Plasma 11beta-Hydroxyprogesterone concentrations does not vary significantly as a function of age, sex, or phase of the menstrual cycle, in contrast to 17-hydroxyprogesterone. Increased plasma 11beta-Hydroxyprogesterone levels in late-onset adrenal 21-hydroxylase deficiency suggest a mild defect of the mineralocorticoid pathway. 21-hydroxylase deficiency (OMIM 201910) is probably the most frequent (if not the most frequent) autosomal recessive genetic disease, occurring in almost 1% of Caucasians and about 3% of Ashkenazi Jews. 21-hydroxylase deficiency is unusual among genetic diseases in that approximately 95% of the mutant alleles have apparently been generated by recombination between a normally active gene (CYP21) and a closely linked pseudogene (CYP21P). There are 4 recognized clinical forms of congenital adrenal hyperplasia, the majority of cases being associated with 21-hydroxylase deficiency: salt-wasting (SW), simple virilizing (SV), nonclassic (NC) late-onset (also called attenuated and acquired), and cryptic. (PMID: 3546944, 2537337)