Detects a 190kDa band in PC-3 cells, which may correspond to Huntingtin. Stains neuronal cytoplasm in immunohistochemistry (the expected localisation). The antibody has not been conclusively tested for specificity for phospho S421 Huntingtin.
Predicted to work with:
Synthetic peptide: RSRSGpSIVE, corresponding to amino acids 416-424 of Human Huntingtin
Fast track antibodies constitute a diverse group of products that have been released to accelerate your research, but are not yet fully characterized. They have all been affinity purified and show high titre values against the immunizing peptide (by ELISA).
Use at an assay dependent concentration. This antibody gave a positive result in ELISA against the immunizing peptide .
May play a role in microtubule-mediated transport or vesicle function.
Expressed in the brain cortex (at protein level). Widely expressed with the highest level of expression in the brain (nerve fibers, varicosities, and nerve endings). In the brain, the regions where it can be mainly found are the cerebellar cortex, the neocortex, the striatum, and the hippocampal formation.
Involvement in disease
Defects in HTT are the cause of Huntington disease (HD) [MIM:143100]. HD is an autosomal dominant neurodegenerative disorder characterized by involuntary movements (chorea), general motor impairment, psychiatric disorders and dementia. Onset of the disease occurs usually in the third or fourth decade of life and symptoms progressively worsen leading to death in 10 to 20 years. Onset and clinical course depend on the degree of poly-Gln repeat expansion, longer expansions resulting in earlier onset and more severe clinical manifestations. HD affects 1 in 10,000 individuals of European origin. Neuropathology of Huntington disease displays a distinctive pattern with loss of neurons, especially in the caudate and putamen (striatum).
Belongs to the huntingtin family. Contains 10 HEAT repeats.
The N-terminal Gln-rich and Pro-rich domain has great conformational flexibility and is likely to exist in a fluctuating equilibrium of alpha-helical, random coil, and extended conformations.
Cleaved by apopain downstream of the polyglutamine stretch. The resulting N-terminal fragment is cytotoxic and provokes apoptosis. Forms with expanded polyglutamine expansion are specifically ubiquitinated by SYVN1, which promotes their proteasomal degradation.
Cytoplasm. Nucleus. The mutant Huntingtin protein colocalizes with AKAP8L in the nuclear matrix of Huntington's disease neurons.
ab2174 was used at a 1:100 dilution to detect phosphorylated Huntingtin by immunohistochemistry in human brain cerebellum. Positive cytoplasmic staining is
observed in neurons. Tissue was formalin-fixed and
paraffin embedded. Detection was with AEC (pink), nuclear counterstaining with Haemotoxylin (blue).