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MAB2166

Sigma-Aldrich

Anti-Huntingtin Protein Antibody, a.a. 181-810, clone 1HU-4C8

ascites fluid, clone 1HU-4C8, Chemicon®

Synonym(s):

Huntingtin, Huntington′s Disease Protein, HD Protein

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About This Item

UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41

biological source

mouse

Quality Level

antibody form

ascites fluid

antibody product type

primary antibodies

clone

1HU-4C8, monoclonal

species reactivity

rat, rabbit

species reactivity (predicted by homology)

mouse, human, hamster, monkey

manufacturer/tradename

Chemicon®

technique(s)

ELISA: suitable
immunocytochemistry: suitable
immunohistochemistry: suitable (paraffin)
immunoprecipitation (IP): suitable
western blot: suitable

isotype

IgG1κ

NCBI accession no.

UniProt accession no.

shipped in

dry ice

target post-translational modification

unmodified

Gene Information

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.

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Storage Class Code

12 - Non Combustible Liquids

WGK

WGK 2

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable


Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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Persistence of morning anticipation behavior and high amplitude morning startle response following functional loss of small ventral lateral neurons in Drosophila.
Sheeba, V; Fogle, KJ; Holmes, TC
Testing null
Huntingtin aggregation kinetics and their pathological role in a Drosophila Huntington's disease model.
Weiss, KR; Kimura, Y; Lee, WC; Littleton, JT
Genetics null
Spatial and temporal requirements for huntingtin (Htt) in neuronal migration and survival during brain development.
Tong, Y; Ha, TJ; Liu, L; Nishimoto, A; Reiner, A; Goldowitz, D
The Journal of Neuroscience null
Localization of BDNF mRNA with the Huntington's disease protein in rat brain.
Ma, B; Culver, BP; Baj, G; Tongiorgi, E; Chao, MV; Tanese, N
Mol. Neurodegener. null
Jiaxin Hu et al.
Nature biotechnology, 27(5), 478-484 (2009-05-05)
Expanded trinucleotide repeats cause many neurological diseases. These include Machado-Joseph disease (MJD) and Huntington's disease (HD), which are caused by expanded CAG repeats within an allele of the ataxin-3 (ATXN3) and huntingtin (HTT) genes, respectively. Silencing expression of these genes

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