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MAB5374

Sigma-Aldrich

Anti-Huntingtin Protein Antibody, clone mEM48

culture supernatant, clone mEM48, Chemicon®

Synonim(y):

Huntingtin

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About This Item

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

pochodzenie biologiczne

mouse

Poziom jakości

forma przeciwciała

culture supernatant

rodzaj przeciwciała

primary antibodies

klon

mEM48, monoclonal

reaktywność gatunkowa

human

reaktywność gatunkowa (przewidywana na podstawie homologii)

mouse, rat

producent / nazwa handlowa

Chemicon®

metody

immunocytochemistry: suitable
immunohistochemistry: suitable
western blot: suitable

izotyp

IgG

numer dostępu NCBI

numer dostępu UniProt

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

Reacts with human huntingtin protein (both native and recombinant protein). MAB5374 reacts with mutant huntingtin in patients and in transgenic animals that express different numbers of repeats (from 82 to 150 glutamines). Thus, it should recognize different forms of mutant huntingtin.

Immunogen

GST fusion protein from the first 256 amino acids from human huntingtin with the deletion of the polyglutamine tract.

Zastosowanie

Detect Huntingtin Protein using this Anti-Huntingtin Protein Antibody, clone mEM48 validated for use in IC, IH & WB.
Immunohistochemistry:
1:50-1:100 of a previous lot using ABC on 4% paraformaldehyde fixed tissue. Suggested dilution buffer is PBS containing 3% BSA. The antibody works on paraffin embedded tissue sections.
Suggested dilution buffer is PBS containing 3% BSA. The antibody works on paraffin embedded tissue sections. Yu, Z et al (2002) Hum. Mole. Genetics 11(8):905-914. (http://hmg.oxfordjournals.org/cgi/content/full/11/8/905) for good IHC methods and photos of mEM48 on rodent tissues with human transgenic material.

Immunocytochemistry:
light 4% PFA fixation followed by 0.1% triton X-100 incubation prior to blocking is suggested.
A previous lot of this antibody was used in IC.

Western blot:
1:50-1:500 using ECL depending on the level of mutant protein. Suggested dilution buffer is PBS containing 3% BSA or PBS containing 5% non-fat milk.
Nuclear fraction preparations enhance signals; monomeric protein ~80kDa; aggregates are common which can be >200kDa in size.

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 HEK293 lysates.

Western Blot Analysis:
1:1000 dilution of this lot detected Huntingtin Protein on 10 μg of HEK293 lysates.

Opis wartości docelowych

over 200 kDa

Postać fizyczna

Culture Supernatant mouse monoclonal IgG containing no preservative.
Unpurified

Przechowywanie i stabilność

Stable for 6 months 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, mouse brain cortex samples from HD or wild type mice

HEK293 lysates.

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)

Poszukaj Certyfikaty analizy (CoA), wpisując numer partii/serii produktów. Numery serii i partii można znaleźć na etykiecie produktu po słowach „seria” lub „partia”.

Masz już ten produkt?

Dokumenty związane z niedawno zakupionymi produktami zostały zamieszczone w Bibliotece dokumentów.

Odwiedź Bibliotekę dokumentów

RNAi screening in Drosophila cells identifies new modifiers of mutant huntingtin aggregation.
J Doumanis, K Wada, Y Kino, AW Moore, N Nukina
Testing null
Viral delivery of glial cell line-derived neurotrophic factor improves behavior and protects striatal neurons in a mouse model of Huntington's disease.
McBride, Jodi L, et al.
Proceedings of the National Academy of Sciences of the USA, 103, 9345-9350 (2006)
Characterization of huntingtin pathologic fragments in human Huntington disease, transgenic mice, and cell models
Schilling, Gabriele, et al
Journal of Neuropathology and Experimental Neurology, 66, 313-320 (2007)
Acute polyglutamine expression in inducible mouse model unravels ubiquitinproteasome system impairment and permanent recovery attributable to aggregate formation.
Ortega Z, D?-az-Hern?!ndez M, Maynard CJ, Hern?!ndez F, Dantuma NP, Lucas JJ
The Journal of Neuroscience null
Willeke M C van Roon-Mom et al.
Neuroreport, 17(6), 667-670 (2006-04-11)
Insoluble protein aggregates have been considered a pathological hallmark of Huntington's disease and other polyglutamine disorders. In this study the number of aggregates was assessed in the superior frontal gyrus and motor cortex of seven Huntington's disease patients and was

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