HD (AA 81-191) antibody

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AA 81-191
Clonality (Clone)
Monoclonal ()
ELISA, Western Blotting (WB)
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Immunogen HD (NP_002102, 81 a.a. ~ 191 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 K
Clone 4G6
Isotype IgG2a kappa
Specificity HD (NP_002102, 81 a.a. ~ 191 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Purification This antibody is purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
Background Synonyms: Serine protease inhibitor J6, 47 kDa heat shock protein, Collagen-binding protein, Colligin, Serpin H1,Hsp47, Cbp1, Serpinh1
Molecular Weight 347860 DA
Gene ID 12406
NCBI Accession NP_002102
Research Area Translation Factors, Transcription Factors, Signaling, Cell Structure
Application Notes ELISA ~~ 1ug/ml~3ng/ml Western blot ~~ 1:500~1000

Background: 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.

Restrictions For Research Use only
Buffer PBS with 0.09% (w/v) sodium azide.
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage 4 °C/-20 °C
Storage Comment Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles
Expiry Date 6 months
Supplier Images
ELISA image for anti-HD (AA 81-191) antibody (ABIN394366) Detection limit for recombinant GST tagged HD is approximately 0.3 ng/mL as a capture...
Background publications Aziz, Pijl, Frölich, Snel, Streefland, Roelfsema, Roos: "Systemic energy homeostasis in Huntington's disease patients." in: Journal of neurology, neurosurgery, and psychiatry, Vol. 81, Issue 11, pp. 1233-7, 2010 (PubMed).

Godin, Poizat, Hickey, Maschat, Humbert: "Mutant huntingtin-impaired degradation of beta-catenin causes neurotoxicity in Huntington's disease." in: The EMBO journal, Vol. 29, Issue 14, pp. 2433-45, 2010 (PubMed).

Olshina, Angley, Ramdzan, Tang, Bailey, Hill, Hatters: "Tracking mutant huntingtin aggregation kinetics in cells reveals three major populations that include an invariant oligomer pool." in: The Journal of biological chemistry, Vol. 285, Issue 28, pp. 21807-16, 2010 (PubMed).

Rose, Behm, Drgon, Johnson, Uhl: "Personalized smoking cessation: interactions between nicotine dose, dependence and quit-success genotype score." in: Molecular medicine (Cambridge, Mass.), Vol. 16, Issue 7-8, pp. 247-53, 2010 (PubMed).

Perlis, Smoller, Mysore, Sun, Gillis, Purcell, Rietschel, Nöthen, Witt, Maier, Iosifescu, Sullivan, Rush, Fava, Breiter, Macdonald, Gusella: "Prevalence of incompletely penetrant Huntington's disease alleles among individuals with major depressive disorder." in: The American journal of psychiatry, Vol. 167, Issue 5, pp. 574-9, 2010 (PubMed).