GeneTex
United States (US)

Huntingtin (neoepitope 552) antibody [4-13]

GTX30673_2080_WB_19011519_794.jpg

Cat No. GTX30673

Host Mouse
Clonality Monoclonal
Clone Name 4-13
Isotype IgG
Application WB, ICC/IF, IHC
Reactivity Human, Mouse
APPLICATION

Application Note

*Optimal dilutions/concentrations should be determined by the researcher.
Application Dilution
WB 1:500 - 1:1000
ICC/IF 2.5 μg/ml
IHC 2.5 μg/ml
Not tested in other applications.

Calculated MW

348 kDa. ( Note )

Specificity/Sensitivity

This antibody recognizes the 552 cleaved fragment without detecting the full-length form.
PROPERTIES

Form

Liquid

Buffer

PBS, 1mg/ml BSA, 0.05% sodium azide

Storage

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.

Concentration

1mg/ml(Please refer to the vial label for the specific concentration.)

Antigen Species

Human

Immunogen

Synthetic peptide conjugated to KLH via cysteine corresponding to residues CSDPAMDLND (544-552) of Human HTT.

Purification

Purified by affinity chromatography

Conjugation

Unconjugated

Note

For laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.
TARGET

Synonyms

HD,IT15,LOMARS

Cellular Localization

Cytoplasm,Nucleus

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. [provided by RefSeq, Jul 2016]

Database

Research Area

DATA IMAGES
GTX30673_2080_WB_19011519_794.jpg

GTX30673 WB Image

WB analysis of over-expressed recombinant HTT fragment lysates (Lane 1) or transfected vector only control lysate (Lane 2) using GTX30673 Huntingtin (neoepitope 552) antibody [4-13].
Dilution : 1:500
Loading: 20 µg

Package List Price ($)
$ 319