*Optimal dilutions/concentrations should be determined by the researcher.
This antibody detects a ~350 kDa band and smaller degradation products of huntingtin.
Increased cytoplasmic staining, relative to nuclear, has been reported using formaldehyde as a fixative compared with acetone/methanol, see Wilkinson et al.
Not tested in other applications.
Clone HDC8A4 reacts with an epitope corresponding to the HDC region (2703 - 2911 amino acids) of the huntingtin protein. This antibody recognizes both denatured and native huntingtin in human brain.
PBS, 0.09% Sodium Azide
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
1.0mg/ml(Please refer to the vial label for the specific concentration.)
Recombinant protein corresponding to amino acids 2703 - 2911 of huntingtin.
Protein G purified
For laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.
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 huntingtin gene is widely expressed and is required for normal development. It is expressed as 2 alternatively polyadenylated forms displaying different relative abundance in various fetal and adult tissues. The larger transcript is approximately 13.7 kb and is expressed predominantly in adult and fetal brain whereas the smaller transcript of approximately 10.3 kb is more widely expressed. 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 huntingtin-associated protein-1, highly expressed in brain, which has increased affinity for huntingtin protein with expanded polyglutamine repeats. This gene contains an upstream open reading frame in the 5' UTR that inhibits expression of the huntingtin gene product through translational repression. [provided by RefSeq, Jul 2008]
GTX31046 WB Image
WB analysis of normal human cerebral cortex tissue lysate using GTX31046 Huntingtin antibody [HDC8A4].