ARG41898

anti-Huntingtin antibody

Datasheet

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100 μl

389.00

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Datasheet

Overview

Product Description	Rabbit Polyclonal antibody recognizes Huntingtin
Tested Reactivity	Hu, Ms, Rat
Tested Application	FACS, ICC/IF, IHC-P, WB
Host	Rabbit
Clonality	Polyclonal
Isotype	IgG
Target Name	Huntingtin
Antigen Species	Human
Immunogen	Synthetic peptide of Human Huntingtin.
Conjugation	Un-conjugated
Alternate Names	Huntingtin; Huntington disease protein; HD protein; IT15; HD

Application Instructions

Application Suggestion

Tested Application	Dilution
FACS	1:100
ICC/IF	1:100 - 1:500
IHC-P	1:50 - 1:200
WB	1:500 - 1:2000

Application Note

* The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist.

Positive Control

HeLa

Properties

Form	Liquid
Purification	Affinity purified.
Buffer	PBS (pH 7.4), 150 mM NaCl, 0.02% Sodium azide and 50% Glycerol.
Preservative	0.02% Sodium azide
Stabilizer	50% Glycerol
Storage Instruction	For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use.
Note	For laboratory research only, not for drug, diagnostic or other use.

Bioinformation

Database Links	GeneID: 3064 Human HTT Swiss-port # P42858 Human Huntingtin
Gene Symbol	HTT
Gene Full Name	huntingtin
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. [provided by RefSeq, Jul 2008]
Function	May play a role in microtubule-mediated transport or vesicle function. [UniProt]
Cellular Localization	Cytoplasm. Nucleus. Note=The mutant Huntingtin protein colocalizes with AKAP8L in the nuclear matrix of Huntington disease neurons. Shuttles between cytoplasm and nucleus in a Ran GTPase-independent manner. [UniProt]
Calculated MW	348 kDa
PTM	Cleaved by apopain downstream of the polyglutamine stretch. The resulting N-terminal fragment is cytotoxic and provokes apoptosis. Forms with expanded polyglutamine expansion are specifically ubiquitinated by SYVN1, which promotes their proteasomal degradation. Phosphorylation at Ser-1179 and Ser-1199 by CDK5 in response to DNA damage in nuclei of neurons protects neurons against polyglutamine expansion as well as DNA damage mediated toxicity. [UniProt]