Technical Data
H7965-01D
Huntingtin Protein (Huntington Disease)
Description:
Huntingtin (Huntington Disease) is the protein product of a disease gene linked to Huntington’s disease, a neuro-degenerative disorder characterized by loss of striatal neurons. This may be caused by an expanded, unstable trinucleotidee repeat in the huntingtin gene, which translates as a polyglutamine repeat in the protein product. The huntingtin gene locus is large, spanning 180kp and consisting of 67 exons. It is expressed as 2 alternatively polyadenylated forms displaying different relative abundance in various fetal and adult tissues. The genetic defect leading to Huntington’s disease may not necessarily eliminate transcription, but may confer a new property on the mRNA or alter the function of the protein. One candidate is the hungtingtin-associated protein-1, highly expressed in the brain, which has increased affinity for huntingtin protein with expanded polyglutamine repeats. Normal huntingtin protein shows a cytoplasmic localization. This protein is widely expressed with the highest level of expression in the brain (nerve fibers, varicosities, nerve endings). In the brain, the regions where it can be mainly found are the cerebellar cortex, the neocortex, the striatum and the hippocampal formation.

Applications:
Suitable for use in ELISA, Western Blot and Immunohistochemistry. Other applications not tested.

Recommended Dilutions:
ELISA: 1:10,000-1:40,000
Western Blot: 1:500-1:3000
Immunohistochemistry (Paraffin): 10-20ug/ml; Tested on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with H7965-01D, slides were incubated with biotinylated secondary antibody, followed by alkaline phosphatase-streptavidin and chromogen.
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. 
TypeIsotypeCloneGrade
PabIgGAffinity Purified
SizeStorageShippingSourceHost
50ug-20°CBlue IceHumanRabbit
Concentration:
~0.5mg/ml
Immunogen:
Synthetic peptide corresponding to aa416-424 of human huntingtin protein phorphorylated at Ser421 (KLH).
Purity:
Purified by immunoaffinity chromatography.
Form
Supplied as a liquid in PBS, pH 7.2, 0.01% sodium azide.
Specificity:
Recognizes the human Huntingtin Protein. Species Sequence Homology: mouse and rat
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.