SerpinA6

Corticosteroid-binding globulin (CBG), also known as SerpinA6, is a non-inhibitory member of the serine proteinase inhibitor (serpin) superfamily. It is the high-affinity transport protein for glucocorticoids in vertebrate blood. CBG is specifically cleaved by this protease at a precise site close to its carboxy-terminus. This induces a conformation change and disrupts the binding between glucocorticoids and CBG, and promotes a significant and local release of glucocorticoids (over 90% of them are bound to CBG in human plasma). In this context, CBG directs glucocorticoids to sites of inflammation, and plays in consequence a crucial role in efficient glucocorticoid action in physiology. The SerpinA6 protein is mainly secreted by the liver. This negative acute phase protein regulates free cortisol levels in the blood and distributes cortisol to its target tissues. SerpinA6 deficiency is an 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. There are three heritable, human CBG gene mutations that can reduce CBG-cortisol binding affinity and/or reduce circulating CBG levels.

SerpinA6 Related Areas

Enzyme>>Protease & Regulator>>Serine Protease & Regulator>>Serpin Superfamily>>SerpinA6

SerpinA6 Alternative Names

SerpinA6, CBG, corticosteroid binding globulin [Homo sapiens]

Serpina6, AI265318, AV104445, Cbg, corticosteroid binding globulin [Mus musculus]

Summaries for SerpinA6

Entrez Gene summary for SerpinA6:

SerpinA6 gene encodes an alpha-globulin protein with corticosteroid-binding properties. This is the major transport protein for glucorticoids and progestins in the blood of most vertebrates. SerpinA6 gene localizes to a chromosomal region containing several closely related serine protease inhibitors which may have evolved by duplication events. [provided by RefSeq, Jul 2008]

Wikipedia summary for SerpinA6:

Transcortin, also known as corticosteroid-binding globulin (CBG) or serpin A6 is a protein that in humans is encoded by the SERPINA6 gene. It is an alpha-globulin.

Human SerpinA6 Protein General Information

 

• Protein names: SerpinA6
• Sequence length: 405 AA.
• Domain: Signal
• Sequence similarities: Belongs to the serpin family.
• Post-translational modification: N-glycosylated; binds 5 oligosaccharide chains.
  Glycosylation in position Asn-260 is needed for steroid binding.
• Subcellular location: Secreted.
• Tissue specificity: Plasma; synthesized in liver. Has also been identified in a number of glycocorticoid responsive cells.
• Involvement in disease: Defects in SERPINA6 are a cause of corticosteroid-binding globulin deficiency (CBG deficiency) . CBG deficiency is an 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.

General information above from UniProt

Function for SerpinA6 Protein

UniProtKB:

SerpinA6 is major transport protein for glucocorticoids and progestins in the blood of almost all vertebrate species.

Genatlas:

• SerpinA6 is corticosteroid binding globulin
• SerpinA6 is serine proteinase inhibitor
• SerpinA6 is involved in the regulation of gene activity and chromatin structure

Phenotype Information for SerpinA6

• Gene/Locus: CBG, SERPINA6
• Phenotype: Corticosteroid-binding globulin deficiency

Phenotype Information for SerpinA6 from OMIM (Online Mendelian Inheritance in Man)

Drugs for SerpinA6

Target	Drug Name	Disease	Drug Status
SerpinA6	Alclometasone	Inflammatory diseases	Approved
SerpinA6	Beclomethasone	Asthma and allergic rhinitis	Approved
SerpinA6	Triamcinolone	Allergies	Approved
SerpinA6	Flurandrenolide	Inflammatory diseases	Approved
SerpinA6	Prednisolone	Asthma and rheumatoid arthritis	Approved
SerpinA6	Rimexolone	Eye Inflammation	Approved
SerpinA6	Fluocinonide	Inflammatory diseases	Approved
SerpinA6	Paramethasone	Inflammatory diseases	Approved
SerpinA6	Ciclesonide	Obstructive airway diseases	Approved
SerpinA6	Fluocinolone Acetonide	Inflammatory diseases	Approved
SerpinA6	Flumethasone Pivalate	Contact dermatitis	Approved

Drugs for SerpinA6 from TTD (Therapeutic Targets Database)