Huntingtin Recombinant Antibody

Applications

WB
FCM
IHC-P
IF(ICC)

Reactivity

Human
Mouse
Rat

Overview
Catalog #	bsm-54305r
Product Name	Huntingtin Recombinant Antibody
Applications	WB, FCM, IHC-P, IF(ICC)
Reactivity	Human, Mouse, Rat
Specifications
Conjugation	Unconjugated
Host	Rabbit
Source	Recombinant protein within human Huntingtin aa 1-150
Immunogen Range	1-150/3142
Clonality	Monoclonal
Isotype	IgG
Concentration	1ug/ul
Purification	Purified by Protein A.
Storage Buffer	0.01M TBS (pH 7.4), 1% BSA, 0.02% Proclin 300, and 50% Glycerol
Storage Condition	Store at 4°C for up to 2 weeks. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
Target
Gene ID	3064
Swiss Prot	P42858
Subcellular location	Cytoplasm, Nucleus
Synonyms	Huntingtin; Huntington disease protein; HD protein; HTT; IT15; LOMARS
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 of trinucleotide repeats (9-35) 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. Huntingtin may play a role in microtubule-mediated transport or vesicle function.
Application Dilution
WB	1:300-5000
FCM	1:20-100
IHC-P	1:200-400
IF(ICC)	1:50-200