Huntingtin Monoclonal Antibody (4-13), Invitrogen™

Description

• MA1-058 has been successfully used in Western blot, immunofluorescence, and immunohistochemistry on human and mouse samples
• Neoepitope antibodies distinguish smaller cleaved fragments or processed forms of proteins versus the intact full-length or precursor by using a designed peptide purification process to maximize immunoreactivity to a specific cleavage site
• Human HTT caspase cleavage sites generate fragment-specific forms of the protein
• Caspase-3/7 has been shown to generate cleavage sites at animo acids 513 and 552
• Caspase-2 cleaves at amino acid 552 and caspase-6 at amino acid 586
• Neo-specific antibody MA1-058 recognizes the 552 cleaved fragment without detecting the full-length form
• Huntingtin is a disease gene linked to Huntington's disease (HD), a neurodegenerative disorder characterized by loss of striatal neurons
• This is thought to be caused by an expanded, unstable trinucleotide repeat in the huntingtin gene, which translates as a polyglutamine repeat in the protein product
• HD is a mid-life onset autosomal dominant neurodegenerative disease that is characterized by psychiatric disorders, dementia, and involuntary movements (chorea), leading to death in 10-20 years.The huntingtin locus is large, spanning 180 kb and consisting of 67 exons
• The huntingtin gene is widely expressed and is required for normal development
• It is expressed as 2 alternatively polyadenylated forms displaying different relative abundance in various fetal and adult tissues
• The larger transcript is approximately 13.7 kb and is expressed predominantly in adult and fetal brain whereas the smaller transcript of approximately 10.3 kb is more widely expressed
• The genetic defect leading to Huntington's disease may not necessarily eliminate transcription, but may confer a new property on the mRNA or alter the function of the protein.