Principle of the Assay: The ELISA is based on the competitive binding enzyme immunoassay technique. The microtiter plate provided in this kit has been pre -coated with an antibody specific to Huntingtin, during the reaction, Huntingtin in the sample or standard competes with a fixed amount of biotin-labeled Huntingtin for sites on a pre-coated Monoclonal antibody specific to Huntingtin. Excess conjugate and unbound sample or standard are washed from the plate. Next, avidin-conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. Then a TMB substrate solution is added to each well. The enzyme-substrate reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The concentration of Huntingtin in the samples is then determined by comparing the O.D. of the samples to the standard curve.
NCBI and Uniprot Product Information
NCBI Description
Huntingtin is a disease gene linked to Huntington's disease, 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. A fairly broad range of trinucleotide repeats (9-35) has been identified in normal controls, and repeat numbers in excess of 40 have been described as pathological. 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. One candidate is the huntingtin-associated protein-1, highly expressed in brain, which has increased affinity for huntingtin protein with expanded polyglutamine repeats. This gene contains an upstream open reading frame in the 5' UTR that inhibits expression of the huntingtin gene product through translational repression. [provided by RefSeq, Jul 2016]
Uniprot Description
Huntingtin: may play a role in microtubule-mediated transport or vesicle function. 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. Defects are the cause of Huntington's disease, 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. 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 isoforms displaying different relative abundance in various fetal and adult tissues.
Protein type: Cytoskeletal
Chromosomal Location of Human Ortholog: 4p16.3
Cellular Component: autophagic vacuole; axon; centriole; cytoplasm; cytoplasmic vesicle membrane; cytosol; dendrite; endoplasmic reticulum; Golgi apparatus; inclusion body; late endosome; nucleoplasm; nucleus; perinuclear region of cytoplasm; protein complex
Molecular Function: beta-tubulin binding; dynein intermediate chain binding; heat shock protein binding; identical protein binding; kinase binding; p53 binding; profilin binding; protein binding; transcription factor binding
Biological Process: establishment of mitotic spindle orientation; Golgi organization and biogenesis; organ development; positive regulation of apoptosis; positive regulation of flagellum biogenesis; positive regulation of inositol-1,4,5-triphosphate receptor activity; protein destabilization; regulation of phosphoprotein phosphatase activity; retrograde vesicle-mediated transport, Golgi to ER; vesicle transport along microtubule; vocal learning
Disease: Huntington Disease
Research Articles on HTT
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Product Notes
The Human HTT htt (Catalog #AAA9426706) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9426706 ELISA Kit recognizes Human HTT. It is sometimes possible for the material contained within the vial of "Huntingtin, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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