Description
Product Usage Information
Western Blotting: 1:1000
Immunoprecipitation: 1:50
Storage
Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/ml BSA and 50% glycerol. Store at -20°C. Do not aliquot the antibody.
Protocol
Available protocols: Western Blotting, Immunoprecipitation
Specificity / Sensitivity
Huntingtin Antibody detects endogenous levels of total huntingtin protein.
Species Reactivity: Human, Mouse, Rat
Source / Purification
Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Pro1218 of human huntingtin protein. Antibodies are purified by protein A and peptide affinity chromatography.
Background
Huntington's Disease (HD) is a fatal neurodegenerative disorder characterized by psychiatric, cognitive, and motor dysfunction. HD neuropathology involves the selective degeneration of specific neuronal subpopulations, including GABA-ergic neurons of the striatum and those within the cerebral cortex (1,2). The genetic analysis of HD has been the flagship study of inherited neurological diseases, from initial chromosomal localization to identification of the gene. Huntingtin is a large (340-350 kDa) cytosolic protein that may be involved in a number of cellular functions such as transcription, gastrulation, neurogenesis, neurotransmission, axonal transport, neural positioning, and apoptosis (2,3). The gene from unaffected individuals contains between 6 and 34 CAG trinucleotide repeats, with expansion beyond this range causing the onset of disease symptoms. A strong inverse correlation exists between the age of onset in patients and the number of huntingtin gene CAG repeats encoding a stretch of polyglutamine peptides (1,2). The huntingtin protein undergoes numerous post-translational modifications, including phosphorylation, ubiquitination, sumoylation, palmitoylation, and cleavage (2). Phosphorylation of Ser421 by Akt can partially counteract the toxicity that results from the expanded polyglutamine tract. Varying Akt expression in the brain correlates with regional differences in huntingtin protein phosphorylation; this pattern inversely correlates with the regions that are most affected by degeneration in diseased brain (2). A key step in the disease is the proteolytic cleavage of huntingtin protein into amino-terminal fragments that contain expanded glutamine repeats and translocate into the nucleus. Caspase-mediated cleavage of huntingtin at Asp513 is associated with increased polyglutamine aggregate formation and toxicity. Phosphorylation of Ser434 by CDK5 protects against cleavage (2,3).
Alternate Names
HD; HD protein; HTT; Huntingtin; Huntingtin, myristoylated N-terminal fragment; Huntington disease protein; IT15; LOMARS
