TARDBP Antibody
Description
Product Specs
Product Science Overview
TAR DNA-binding protein 43 (TDP-43) is a crucial RNA-binding protein that plays a significant role in RNA processing and stability. It was initially identified as a transcriptional repressor binding to the TAR DNA element of the HIV-1 virus . TDP-43 has since been shown to bind both RNA and DNA, and it has multiple functions in transcriptional repression, translational regulation, and pre-mRNA splicing .
In 2006, TDP-43 was identified as a major component of ubiquitinated inclusions in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) . It was found to be mislocalized from the nucleus to the cytoplasm in neurons and glia in affected central nervous system regions . Soon after, TDP-43 mutations were discovered in families with inherited ALS and FTD, suggesting a primary role for TDP-43 disruption in disease pathogenesis .
The nuclear clearance and cytoplasmic mislocalization of TDP-43 are pathological hallmarks of ALS, FTD, and related neurodegenerative disorders collectively termed "TDP-43 proteinopathies" . TDP-43 mislocalization causes neurodegeneration through both loss and gain of function mechanisms. Loss of TDP-43 nuclear RNA processing function destabilizes the transcriptome by multiple mechanisms, including disruption of pre-mRNA splicing, failure of repression of cryptic exons, and retrotransposon activation . The accumulation of cytoplasmic TDP-43, which is prone to aberrant liquid–liquid phase separation and aggregation, traps TDP-43 in the cytoplasm and disrupts a host of downstream processes, including the trafficking of RNA granules, local translation within axons, and mitochondrial function .
The mouse anti-human TDP-43 antibody is a monoclonal antibody that specifically detects human TDP-43 in various applications, including Western blot, immunohistochemistry, immunoprecipitation, and immunocytochemistry . This antibody is purified from hybridoma culture supernatant using protein A or G and is available in lyophilized form . It is used in research to study the expression and localization of TDP-43 in human, mouse, and rat tissues .
The mouse anti-human TDP-43 antibody has been widely used in research to investigate the role of TDP-43 in neurodegenerative diseases. It helps in understanding the mechanisms associated with TDP-43 neurotoxicity in ALS and FTD . Researchers use this antibody to study the mislocalization and aggregation of TDP-43 in affected neurons and glia, providing insights into the disease mechanisms and potential therapeutic targets .
