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MAB2166

Sigma-Aldrich

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

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

Synonim(y):

Huntingtin, Huntington′s Disease Protein, HD Protein

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About This Item

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

pochodzenie biologiczne

mouse

Poziom jakości

forma przeciwciała

ascites fluid

rodzaj przeciwciała

primary antibodies

klon

1HU-4C8, monoclonal

reaktywność gatunkowa

rat, rabbit

reaktywność gatunkowa (przewidywana na podstawie homologii)

mouse, human, hamster, monkey

producent / nazwa handlowa

Chemicon®

metody

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

izotyp

IgG1κ

numer dostępu NCBI

numer dostępu UniProt

Warunki transportu

dry ice

docelowa modyfikacja potranslacyjna

unmodified

informacje o genach

Opis ogólny

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.

Specyficzność

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.

Zastosowanie

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

Jakość

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.

Opis wartości docelowych

~ 350-400 kDa

Postać fizyczna

Ascites mouse monoclonal IgG1κ liquid containing no preservative
Unpurified

Przechowywanie i stabilność

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.

Komentarz do analizy

Control
Normal human cerebral cortex lysate

Inne uwagi

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

Informacje prawne

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

Oświadczenie o zrzeczeniu się odpowiedzialności

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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Kod klasy składowania

12 - Non Combustible Liquids

Klasa zagrożenia wodnego (WGK)

WGK 2

Temperatura zapłonu (°F)

Not applicable

Temperatura zapłonu (°C)

Not applicable


Certyfikaty analizy (CoA)

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Tailor-made RNAi knockdown against triplet repeat disease-causing alleles.
Takahashi, M; Watanabe, S; Murata, M; Furuya, H; Kanazawa, I; Wada, K; Hohjoh, H
Proceedings of the National Academy of Sciences of the USA null
C L Benn et al.
Neuroscience, 147(2), 354-372 (2007-06-05)
A yeast artificial chromosome (YAC) mouse model of Huntington's disease (YAC128) develops motor abnormalities, age-dependent striatal atrophy and neuronal loss. Alteration of neurotransmitter receptors, particularly glutamate and dopamine receptors, is a pathological hallmark of Huntington's disease. We therefore analyzed neurotransmitter
Co-localization of brain-derived neurotrophic factor (BDNF) and wild-type huntingtin in normal and quinolinic acid-lesioned rat brain
Fusco, F.R. et al.
The European Journal of Neuroscience, 18, 1-10 (2003)
L Cai et al.
Neuroscience, 181, 196-205 (2011-03-15)
The transcription regulator, neuron-restrictive silencer factor (NRSF), also known as repressor element-1 silencing transcription factor (REST), plays an important role in neurogenesis and various neuronal diseases such as ischaemia, epilepsy, and Huntington's disease. In these disease processes, neuronal loss is
M O Hebb et al.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 13(9), 1099-1106 (1999-05-25)
Huntington's disease (HD) is one of a number of neurodegenerative disorders caused by expansion of polyglutamine-encoding CAG repeats within specific genes. Huntingtin, the protein product of the HD gene, is widely expressed in neural and nonneural human and rodent tissue.

Produkty

Immunofluorescencja wykorzystuje cząsteczki fluorescencyjne sprzężone z przeciwciałami do lokalizacji białek, potwierdzania modyfikacji i wizualizacji kompleksów białkowych.

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