HSD11B2 Chromosome 16

Hydroxysteroid 11-beta dehydrogenase 2
20 variants 20 Health Risk

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What This Gene Does
There are at least two isozymes of the corticosteroid 11-beta-dehydrogenase, a microsomal enzyme complex responsible for the interconversion of cortisol and cortisone. The type I isozyme has both 11-beta-dehydrogenase (cortisol to cortisone) and 11-oxoreductase (cortisone to cortisol) activities. The type II isozyme, encoded by this gene, has only 11-beta-dehydrogenase activity. In aldosterone-selective epithelial tissues such as the kidney, the type II isozyme catalyzes the glucocorticoid cortisol to the inactive metabolite cortisone, thus preventing illicit activation of the mineralocorticoid receptor. In tissues that do not express the mineralocorticoid receptor, such as the placenta and testis, it protects cells from the growth-inhibiting and/or pro-apoptotic effects of cortisol, particularly during embryonic development. Mutations in this gene cause the syndrome of apparent mineralocorticoid excess and hypertension. [provided by RefSeq, Feb 2010]
Gene Info
Gene Group
Short chain dehydrogenase/reductase superfamily
Locus Type
gene with protein product
Location
16q22.1
Ensembl
ENSG00000176387
Associated Conditions (4)
Apparent mineralocorticoid excess
HSD11B2-related disorder
Inborn genetic diseases
mild
Key Variants
All Variants (20)
RSID Category Clinical Significance Conditions
RS121917833 Health Risk Conflicting classifications of pathogenicity Apparent mineralocorticoid excess, Apparent mineralocorticoid excess
RS1555518481 Health Risk Conflicting classifications of pathogenicity Apparent mineralocorticoid excess, HSD11B2-related disorder, Apparent mineralocorticoid excess
RS45619232 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Apparent mineralocorticoid excess, Inborn genetic diseases
RS765574993 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS794726669 Health Risk Conflicting classifications of pathogenicity Apparent mineralocorticoid excess, mild, Apparent mineralocorticoid excess
RS1453036708 Health Risk Likely pathogenic Apparent mineralocorticoid excess, Apparent mineralocorticoid excess
RS768507002 Health Risk Likely pathogenic
RS121917780 Health Risk Pathogenic Apparent mineralocorticoid excess, Apparent mineralocorticoid excess
RS121917781 Health Risk Pathogenic Apparent mineralocorticoid excess, Apparent mineralocorticoid excess
RS121917782 Health Risk Pathogenic Apparent mineralocorticoid excess, mild, Apparent mineralocorticoid excess
RS1472555983 Health Risk Pathogenic
RS2040931363 Health Risk Pathogenic
RS2040932336 Health Risk Pathogenic
RS2040979235 Health Risk Pathogenic Apparent mineralocorticoid excess, Apparent mineralocorticoid excess
RS28934591 Health Risk Pathogenic Apparent mineralocorticoid excess, Apparent mineralocorticoid excess
RS28934594 Health Risk Pathogenic Apparent mineralocorticoid excess, Apparent mineralocorticoid excess
RS387907117 Health Risk Pathogenic Apparent mineralocorticoid excess, Apparent mineralocorticoid excess
RS794726684 Health Risk Pathogenic Apparent mineralocorticoid excess, Apparent mineralocorticoid excess
RS28934592 Health Risk Pathogenic/Likely pathogenic Apparent mineralocorticoid excess, Apparent mineralocorticoid excess
RS397509434 Health Risk Pathogenic/Likely pathogenic Apparent mineralocorticoid excess, Apparent mineralocorticoid excess
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