General description
Huntington disease (HD) is a hereditary, progressive, neurodegenerative ailment characterized by personality changes, motor impairment and subcortical dementia. The molecular basis of the disease involves the expansion of the trinucleotide CAG, coding for polyglutamine in the first exon of a chromosome four gene (4p16.3), which normally produces a widely expressed 3136 a.a. (~350 kDa) protein huntingtin with unclear function. The protein is found in the perinuclear region along with microtubules, and in the centrosomal region along with gamma-tubulin. Huntingtin is necessary for neuronal survival and is involved in synaptic vesicle trafficking, microtubule binding and may also have a role in apoptosis. In the HD condition, neuronal cells with the mutant form of huntingtin possess intranuclear aggregations of the N-terminal fragment, causing damaging inclusions in perinuclear locations and striatal neuron cell death. Wild-type huntington and anti-huntingtin reduce aggregation and cellular toxicity of the mutant huntingtin form in mammalian cell models of HD. Huntingtin is known to interact with GAPDH, HAP-1, SP1 and TAFII130.
Specificity
Huntingtin Protein. No detectable cross reactivity to other proteins by Western blot.
Immunogen
Epitope: a.a. 181-810
Huntingtin fragment from a.a. 181 to 810 as a fusion protein.
Application
Anti-Huntingtin Protein Antibody, a.a. 181-810, clone 1HU-4C8 is an antibody against Huntingtin Protein for use in ELISA, IC, IH(P), IP & WB.
ELISA:
A 1:500-1:5,000 dilution of a previous lot was used on ELISA.
Immunohistochemistry:
A 1:500-1:5,000 dilution from a previous lot was used on frozen and microwave oven treated paraffin sections (human tissue).
Immunocytochemistry:
1:500-1:5,000 on a previous lot was used on transfected cells.
Immunoprecipitation:
A 1:500-1:5,000 dilution of a previous lot was used on immunoprecipitation.
Western blot:
1:500-1:5,000. Should detect a band migrating at approximately 350-400 kDa by Western blot (Nature Genetics 10:104-110.).
Optimal working dilutions must be determined by the end user.
Research Category
Neuroscience
Research Sub Category
Neurodegenerative Diseases
Quality
Routinely evaluated by Western Blot on rat brain lysates.
Western Blot Analysis:
1:1000 dilution of this lot detected huntingtin protein on 10 μg of rat brain lysates.
Target description
~ 350-400 kDa
Physical form
Ascites mouse monoclonal IgG1κ liquid containing no preservative
Unpurified
Storage and Stability
Stable for 1 year at -20ºC in undiluted aliquots from date of receipt.
Handling Recommendations: Upon receipt, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.
Analysis Note
Control
Normal human cerebral cortex lysate
Other Notes
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
Legal Information
CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Shipping Information:
Dry Ice Surcharge & Ice Pack Shipments: $40
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- UPC:
- 12352204
- Condition:
- New
- Availability:
- 3-5 Days
- Weight:
- 1.00 Ounces
- HazmatClass:
- No
- MPN:
- MAB2166
- Temperature Control Device:
- Yes
