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- 英文名：
BioLegend 856302 Purified anti-Transthyretin
- 供应商：
北京云肽生物科技有限公司
BioLegend 856302 Purified anti-Transthyretin
北京云肽生物科技有限公司作为 BioLegend 的特约经销商，为广大客户提供原装正品，货期短，折扣好的服务准则。
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BioLegend 856302 Purified anti-Transthyretin
产品简介：
产品品牌：BioLegend
产品货号：856302
产品名称：BioLegend 856302 Purified anti-Transthyretin
产品规格：100 µg
产品说明：
Clone A16143A (See other available formats)
Regulatory Status RUO
Other Names TTR, ATTR, Prealbumin, TBPA, PALB
Isotype Mouse IgG1, κ
描述
转甲状腺素（TTR）是一种载体蛋白，能够在血浆和脑脊液中运输甲状腺激素，并在血浆中运输视黄醇（维生素a）。TTR可以形成异常的不溶性淀粉样纤维，主要沉积在患者的心脏、外周神经或两者中。该野生型蛋白与老年系统性淀粉样变性（SSA）有关，通常在70岁以上的男性中诊断。TTR的单突变或双突变主要与家族性淀粉样蛋白多发性神经病（FAP）或家族性淀粉状蛋白心脏病（FAC）有关，后者影响年轻患者，通常在30多岁。TTR淀粉样变性的临床症状历来归因于原纤维，但最近的实验证据表明可溶性TTR聚集体也具有致病性。
Transthyretin (TTR) is a carrier protein capable of transporting thyroid hormones in the plasma and cerebrospinal fluid, and retinol (vitamin A) in the plasma. TTR can form aberrant, insoluble amyloid fibrils that primarily deposit in a patient's heart, peripheral nerves, or both. The wild-type protein is associated with senile systemic amyloidosis (SSA) and is typically diagnosed in men over 70-years-old. Single or double mutations in TTR are primarily associated with familial amyloidotic polyneuropathy (FAP) or familial amyloid cardiopathy (FAC) which affects younger patients, often in their 30s. Clinical symptoms of TTR amyloidosis have historically been attributed to fibrils, but recent experimental evidence indicates that soluble TTR aggregates are also pathogenic.
Product Details
Verified Reactivity Human, Mouse, Rat
Antibody Type Monoclonal
Host Species Mouse
Immunogen TTR-KLH peptide
Formulation
Phosphate-buffered solution, pH 7.2, containing 0.09% sodium azide.
Preparation
The antibody was purified by affinity chromatography.
Concentration 0.5 mg/ml
Storage & Handling
The antibody solution should be stored undiluted between 2°C and 8°C.
Application WB - Quality tested
IHC-P - Verified
Recommended Usage
Each lot of this antibody is quality control tested by Western blotting. For Western blotting, the suggested use of this reagent is 1.0 - 10 µg per ml. For immunohistochemistry on formalin-fixed paraffin-embedded tissue, a concentration range of 1.0 - 10 µg/ml is suggested. It is recommended that the reagent be titrated for optimal performance for each application.
Antigen Details
Structure
Transthyretin (TTR) is a β-sheet rich homotetrameric protein that has a molecular mass of 55 kD. Each monomer consists of 147 amino acids.
Distribution
Primarily extracellular in blood and cerebrospinal fluid.
Function
The normal function of TTR is to transport thyroxine and retinol-binding protein in the blood and cerebrospinal fluid.
Interaction
Perlecan, receptor for advanced glycation end product (RAGE).
Cell Sources
Liver, choroid plexus epithelium, retina pigment epithelium.
Biology Area
Cell Biology, Neurodegeneration, Neuroscience, Protein Misfolding and Aggregation
Molecular Family Carrier Proteins
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BioLegend 856302 Purified anti-Transthyretin
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