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Anti-Huntingtin, clone 4A4.2
Recommended Replacement for: MAB5492
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Species Reactivity Key Applications Host Format Antibody Type
H, R WB, IHC Mouse Purified Monoclonal Antibody
Description:
Anti-Huntingtin Antibody, clone 4A4.2
Replaces:
MAB5492
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Molecular Weight:
~348 kDa observed
Immunogen:
Recombinant protein corresponding to human Huntingtin.
Clone:
4A4.2
Isotype:
IgG2aκ
Background Information:
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. Huntingtin is known to interact with GAPDH, HAP-1, SP1 and TAFII130.
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Species Reactivity:
Human
Rat
Species Reactivity Note:
Demonstrated to react with Human and Rat.
Application Notes:
Immunohistochemistry Analysis: 1:400 dilution of this antibody from a representative lot detected Huntingtin in the neurons and glial cells of the normal human brain.
Control:
Rat brain tissue lysate
Quality Assurance:
Evaluated by Western Blot in rat brain tissue lysate.
Western Blot Analysis: 0.5 µg/mL of this antibody detected Huntingtin on 10 µg of rat brain tissue lysate.
Purification Method:
Protein G
Presentation:
Purified mouse monoclonal IgG2aκ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Storage Conditions:
Stable for 1 year at 2-8°C from date of receipt.
UniProt Number:
P42858
Entrez Gene Number:
NP_002102
Gene Symbol:
HTT
HD
IT15
Alternate Names:
Huntingtin
Huntington disease protein
HD protein
Usage Statement:
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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Key Applications:
Western Blotting
Immunohistochemistry
Entrez Gene Summary:
Huntingtin is a disease gene linked to Huntington's disease, a neurodegenerative disorder characterized by loss of striatal neurons. This is thought to be caused by an expanded, unstable trinucleotide repeat in the huntingtin gene, which translates as a polyglutamine repeat in the protein product. A fairly broad range in the number of trinucleotide repeats has been identified in normal controls, and repeat numbers in excess of 40 have been described as pathological. The huntingtin locus is large, spanning 180 kb and consisting of 67 exons. The hu |