HUNTINGTINS DISEASE; MS X-100UL

Code: mab2166 D2-231

Analysis Note

ControlNormal human cerebral cortex lysate

Application

Research Sub CategoryNeurodegenerative Diseases

Research CategoryNeuro...


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Analysis Note

ControlNormal human cerebral cortex lysate

Application

Research Sub CategoryNeurodegenerative Diseases

Research CategoryNeuroscience

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.

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.

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.

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.

Immunogen

Huntingtin fragment from a.a. 181 to 810 as a fusion protein.

Epitope: a.a. 181-810

Legal Information

CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany

Other Notes

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.

Physical form

Ascites mouse monoclonal IgG1κ liquid containing no preservative

Unpurified

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.

Specificity

Huntingtin Protein. No detectable cross reactivity to other proteins by Western blot.

Storage and Stability

Stable for 1 year at -20°C 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.

Target description

~ 350-400 kDa

antibody formascites fluid
antibody product typeprimary antibodies
biological sourcemouse
clone1HU-4C8, monoclonal
Gene Informationhuman ... HTT(3064), SLC6A4(6532)
isotypeIgG1κ
manufacturer/tradenameChemicon®
NCBI accession no.NM_002111.6
Quality Level100
shipped indry ice
species reactivity (predicted by homology)human, monkey, hamster, mouse
species reactivityrat, rabbit
technique(s)immunocytochemistry: suitable, western blot: suitable, immunohistochemistry: suitable (paraffin), immunoprecipitation (IP): suitable, ELISA: suitable
UniProt accession no.P42858
This product has met the following criteria: