Biolegend, 848201, Purified anti-Transthyretin, aggregated Antibody, 25μg

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.
25μg
Verified Reactivity: Human
Antibody Type: Monoclonal
Host Species: Mouse
Immunogen: A 1:1 mixture of wild-type Transthyretin fibrils and soluble aggregates of Y78F TTR.
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: Direct ELISA - Quality tested ELISA Capture, IHC-P - Verified
Recommended Usage: Each lot of this antibody is quality control tested by Direct ELISA. For Direct ELISA, the optimal concentration of this reagent for the specific application and the experiment design should be established by the end-user. For ELISA capture applications, a concentration of 2.0 µg/mL is recommended. For immunohistochemical staining on formalin-fixed paraffin-embedded tissue sections, a concentration of is 1.0 - 5.0 µg/mL is recommmended. It is recommended that the reagent be titrated for optimal performance for each application.
Application Notes: Clone 2T5C9 recognizes a mid-region epitope on transthyretin aggregates.
Application References(PubMed link indicates BioLegend citation): Phay M, et al. 2014. Rejuvenation Res. 17(2):97. (Direct ELISA, Capture ELISA, IHC) PubMed
RRID: AB_2632817 (BioLegend Cat. No. 848201) AB_2632818 (BioLegend Cat. No. 848202)
Structure: Transthyretin (TTR) is a β-sheet rich homotetrameric protein that has a molecular mass of 55 kD. Each monomer consists of 127 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).
Ligand/Receptor: Thyroxine, retinol-binding protein bound to retinol.
Cell Sources: Liver, choroid plexus epithelium, retina pigment epithelium.
Biology Area: Cell Biology, Neurodegeneration, Neuroscience, Protein Misfolding and Aggregation
Antigen References: 1. Plante-Bordeneuve V, Said G. 2011. Lancet Neurol. 10:1086. PubMed 2. Pinney JH, et al. 2013. J. Am. Heart Assoc. 2:e000098. PubMed 3. Hawkins PN, et al. 2015. Ann Med. 47(8):625. PubMed
Gene ID: 7276
UniProt: View information about Transthyretin on UniProt.org
Clone: 2T5C9
Regulatory Status: RUO
Other Names: PALB, ATTR, Prealbumin, TBPA, TTR
Isotype: Mouse IgG1, κ