SerpinA6 抗体 (Y19-E): sc-100851

SerpinA6 Antibody (Y19-E) is an IgG₁ κ mouse monoclonal SerpinA6 antibody (also designated Transcortin antibody or CBG antibody) suitable for the detection of the SerpinA6 protein of human origin by WB, IP and ELISA. SerpinA6 Antibody (Y19-E) is available as the non-conjugated anti-SerpinA6 antibody. The serine proteinase inhibitors (serpins) comprise a superfamily of proteins with a diverse set of functions, including the control of blood coagulation, complement activation, programmed cell death and tissue development. SerpinA6, also known as CBG or Transcortin, is a 405 amino acid α-globulin secreted protein that belongs to the serpin family. Synthesized in the liver and present in glycocorticoid responsive cells, SerpinA6 functions as the primary transport protein for progestins and glucorticoids within the blood. Additionally, SerpinA6 has corticosteroid-binding properties through which it can regulate the physiological binding of serum cortisol within the cell. Defects in the gene encoding SerpinA6 are the cause of corticosteroid-binding globulin deficiency (CBG deficiency), a rare disorder characterized by reduced corticosteroid-binding rates that result in hypo/hypertension and muscle fatigue.

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References:

1. Novel human corticosteroid-binding globulin variant with low cortisol-binding affinity.  |  Emptoz-Bonneton, A., et al. 2000. J Clin Endocrinol Metab. 85: 361-7. PMID: 10634411
2. A Leu----His substitution at residue 93 in human corticosteroid binding globulin results in reduced affinity for cortisol.  |  Smith, CL., et al. 1992. J Steroid Biochem Mol Biol. 42: 671-6. PMID: 1504007
3. Corticosteroid binding globulin gene polymorphism influences cortisol driven fat distribution in obese women.  |  Barat, P., et al. 2005. Obes Res. 13: 1485-90. PMID: 16222046
4. Sequence diversity at the proximal 14q32.1 SERPIN subcluster: evidence for natural selection favoring the pseudogenization of SERPINA2.  |  Seixas, S., et al. 2007. Mol Biol Evol. 24: 587-98. PMID: 17135331
5. Haploinsufficiency of the SERPINA6 gene is associated with severe muscle fatigue: A de novo mutation in corticosteroid-binding globulin deficiency.  |  Buss, C., et al. 2007. J Neural Transm (Vienna). 114: 563-9. PMID: 17245537
6. Corticosteroid-binding globulin gene polymorphisms: clinical implications and links to idiopathic chronic fatigue disorders.  |  Torpy, DJ. and Ho, JT. 2007. Clin Endocrinol (Oxf). 67: 161-7. PMID: 17547679
7. Decreased cortisol-binding affinity of transcortin Leuven is associated with an amino acid substitution at residue-93.  |  Van Baelen, H., et al. 1993. Steroids. 58: 275-7. PMID: 8212073