Phospho-Huntington (Ser421)Rabbit anti-Human, Polyclonal, Rockland™

Description

• Store vial at -20°C prior to opening
• Aliquot contents and freeze at -20°C or below for extended storage
• Avoid cycles of freezing and thawing
• Centrifuge product if not completely clear after standing at room temperature
• This product is stable for several weeks at 4°C as an undiluted liquid
• Dilute only prior to immediate use
• This antibody is specific for phosphorylated human Huntington protein at the pS421 residue
• BLAST analysis indicates 100 % homology of the immunizing sequence with Huntington homologues from chimpanzee, pig and chicken
• Cross reactivity with Huntington protein homologues from mouse and rat may also occur as sequence homology varies by one amino acid residue in this sequence
• Reactivity with Huntington protein from other sources is not known
• Minimal reactivity is expected with the non-phosphorylated form of the protein
• 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.