SERPINA6
Domain
Proteolytic cleavage leads to an important conformation change. This reduces the affinity for steroids.
Function
Major transport protein for glucocorticoids and progestins in the blood of almost all vertebrate species.
Involvement in disease
Corticosteroid-binding globulin deficiency
CBG deficiency
Extremely rare hereditary disorder characterized by reduced corticosteroid-binding capacity with normal or low plasma corticosteroid-binding globulin concentration, and normal or low basal cortisol levels associated with hypo/hypertension and muscle fatigue.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
N-glycosylated; binds 5 oligosaccharide chains.
Glycosylation in position Asn-260 is needed for steroid binding.
Sequence Similarities
Belongs to the serpin family.
Tissue Specificity
Plasma; synthesized in liver. Has also been identified in a number of glycocorticoid responsive cells.
Cellular localization
- Secreted
Alternative names
CBG, SERPINA6, Corticosteroid-binding globulin, Serpin A6, Transcortin