蛋白名称
Transthyretin (ATTR) (Prealbumin) (TBPA)
免疫原
Recombinant Protein of TTR
特异性
The antibody detects endogenous TTR protein
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
IF: 1:50-200 WB 1:500-2000,IHC-p 1:50-300
纯化工艺
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
其他名称
Transthyretin (ATTR) (Prealbumin) (TBPA)
背景
This gene encodes transthyretin, one of the three prealbumins including alpha-1-antitrypsin, transthyretin and orosomucoid. Transthyretin is a carrier protein; it transports thyroid hormones in the plasma and cerebrospinal fluid, and also transports retinol (vitamin A) in the plasma. The protein consists of a tetramer of identical subunits. More than 80 different mutations in this gene have been reported; most mutations are related to amyloid deposition, affecting predominantly peripheral nerve and/or the heart, and a small portion of the gene mutations is non-amyloidogenic. The diseases caused by mutations include amyloidotic polyneuropathy, euthyroid hyperthyroxinaemia, amyloidotic vitreous opacities, cardiomyopathy, oculoleptomeningeal amyloidosis, meningocerebrovascular amyloidosis, carpal tunnel syndrome, etc. [provided by RefSeq, Jan 2009],
功能
disease:Defects in TTR are a cause of hyperthyroxinemia [MIM:176300].,disease:Defects in TTR are the cause of amyloidosis type 1 (AMYL1) [MIM:176300]. AMYL1 is a hereditary generalized amyloidosis due to transthyretin amyloid deposition. Protein fibrils can form in different tissues leading to amyloid polyneuropathies, amyloidotic cardiomyopathy, carpal tunnel syndrome, systemic senile amyloidosis.,disease:Defects in TTR are the cause of amyloidosis type 7 (AMYL7) [MIM:105210]; also known as leptomeningeal amyloidosis or meningocerebrovascular amyloidosis. AMYL7 is a form of hereditary transthyretin amyloidosis characterized by primary involvement of the central nervous system. Neuropathologic examination shows amyloid in the walls of leptomeningeal vessels, in pia arachnoid, and subpial deposits. Some patients also develop vitreous amyloid deposition that leads to visual impairment (ocu
