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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H, M | ELISA, IP, WB, ICC, IH(P) | M | Ascites | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MAB2170 |
| Brand Family | Chemicon® |
| Trade Name |
|
| Description | Anti-Huntingtin Protein Antibody, a.a. 1247-1646, clone HU-4E6 |
| Product Information | |
|---|---|
| Format | Ascites |
| Presentation | Ascites fluid. Liquid, does not contain any preservative. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Detect Huntingtin Protein using this Anti-Huntingtin Protein Antibody, a.a. 1247-1646, clone HU-4E6 validated for use in ELISA, IP, WB, IC, IH(P). |
| Key Applications |
|
| Application Notes | ELISA: 1:500-1:5,000 Western blot (recombinant protein, not suitable for the natural Huntingtin) : 1:500-1:5,000 Immunohistochemistry on frozen and microwave oven treated paraffin sections (human): 1:500-1:5,000 Immunocytochemistry on transfected cells: 1:500-1:5,000 Immunoprecipitation: 1:500-1:5,000 Optimal working dilutions must be determined by the end user. |
| Biological Information | |
|---|---|
| Immunogen | Huntingtin fragment from aa 1247 to 1646 as a fusion protein |
| Epitope | a.a. 1247-1646 |
| Clone | HU-4E6 |
| Host | Mouse |
| Specificity | Huntingtin Protein. No detectable cross reactivity with other proteins by Western blot. |
| Isotype | IgG2b |
| Species Reactivity |
|
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
|
| Entrez Gene Summary | Huntingtin is a disease gene linked to Huntingtons 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 in the number of trinucleotide repeats 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 Huntingtons 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. |
| Gene Symbol |
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| UniProt Number |
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| UniProt Summary | FUNCTION: SwissProt: P42858 # May play a role in microtubule-mediated transport or vesicle function. SIZE: 3144 amino acids; 347860 Da SUBUNIT: Binds SH3GLB1 (By similarity). Interacts through its N- terminus with FNBP3. Interacts with PQBP1, SETD2 and SYVN. SUBCELLULAR LOCATION: Cytoplasm. Nucleus. TISSUE SPECIFICITY: 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. PTM: Cleaved by apopain downstream of the polyglutamine stretch. The resulting amino-terminal fragment is cytotoxic and provokes apoptosis. & Forms with expanded polyglutamine expansion are specifically ubiquitinated by SYVN1, which promotes their proteasomal degradation. DISEASE: SwissProt: P42858 # Defects in HD 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). SIMILARITY: SwissProt: P42858 ## Belongs to the hungtintin family. & Contains 10 HEAT repeats. |
| Product Usage Statements | |
|---|---|
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Maintain at -20°C in undiluted aliquots up to 12 months after date of receipt. Avoid repeated freeze/thaw cycles. During shipment, small volumes of antibody will occasionally become entrapped in the seal of the product vial. For antibodies with volumes of 200 μl or less, we recommend gently tapping the vial on a hard surface or briefly centrifuging the vial in a tabletop centrifuge to dislodge any liquid in the containers cap. |
| Packaging Information | |
|---|---|
| Material Size | 100 µL |