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HTT peptide :: Huntingtin Protein Peptide

Scan QR to view Datasheet Catalog #    MBS658259 HTT peptide
Unit / Price
1 mg  /  $310 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Huntingtin Protein (HTT), Peptide

 Also Known As   

Huntingtin Protein, phosphorylated (Ser421), Control Peptide (Huntington Disease)

 Product Gene Name   

HTT peptide

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Chromosome Location    Chromosome: 4; NC_000004.11 (3076408..3245687). Location: 4p16.3
 OMIM    143100
 3D Structure    ModBase 3D Structure for P42858
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 Species Reactivity    Human
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 Specificity    Blocks specific interaction of anti-Huntingtin with Huntingtin.
 Purity/Purification    Highly Purified
0.8
 Form/Format    Supplied as a lyophilized powder.
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 Preparation and Storage    Lyophilized powder may be stored at 4 degree C for short-term only. Stable for 12 months at -20 degree C. Reconstitute to nominal volume (see reconstitution instructions for peptides) and store at -20 degree C. For maximum recovery of product, centrifuge the original vial prior to removing the cap. Further dilutions can be made in assay buffer.
 Other Notes    Small volumes of HTT peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for HTT peptide

   Control peptide for H7965-03
 Product Categories/Family for HTT peptide    Molecular Biology; MB-Disease Markers
 Applications Tested/Suitable for HTT peptide   

ELISA (EL/EIA)

 Application Notes for HTT peptide    Suitable for use in ELISA.
Dilution: ELISA: 1ug/ 1ul anti-serum.
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NCBI/Uniprot data below describe general gene information for HTT. It may not necessarily be applicable to this product.
 NCBI GI #    90903231
 NCBI GeneID    3064
 NCBI Accession #    NP_002102.4 [Other Products]
 NCBI GenBank Nucleotide #    NM_002111.6 [Other Products]
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 UniProt Primary Accession #    P42858 [Other Products]
 UniProt Secondary Accession #    Q9UQB7 [Other Products]
 UniProt Related Accession #    P42858 [Other Products]
 Molecular Weight    347,603 Da [Similar Products]
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 NCBI Official Full Name    huntingtin
 NCBI Official Synonym Full Names    huntingtin
 NCBI Official Symbol    HTT [Similar Products]
 NCBI Official Synonym Symbols   
HD; IT15
[Similar Products]
 NCBI Protein Information    huntingtin; huntington disease protein
 UniProt Protein Name    Huntingtin
 UniProt Synonym Protein Names   
Huntington disease protein
 Protein Family    HD protein
 UniProt Gene Name    HTT [Similar Products]
 UniProt Synonym Gene Names    HD; IT15; HD protein [Similar Products]
 UniProt Entry Name    HD_HUMAN
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 NCBI Summary for HTT    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]
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 UniProt Comments for HTT    Function: May play a role in microtubule-mediated transport or vesicle function.

Subunit structure: Binds SH3GLB1

By similarity. Interacts through its N-terminus with PRPF40A. Interacts with PQBP1, SETD2 and SYVN. Interacts with PFN1. Interacts with TPR; the interaction is inhibited by forms of Huntingtin with expanded polyglutamine stretch. Ref.9 Ref.10 Ref.11 Ref.12 Ref.14 Ref.16 Ref.20

Subcellular location: 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. Ref.7 Ref.14 Ref.15

Tissue specificity: Expressed in the brain cortex (at protein level). Widely expressed with the highest level of expression in the brain (nerve fibers, varicosities, and nerve endings). In the brain, the regions where it can be mainly found are the cerebellar cortex, the neocortex, the striatum, and the hippocampal formation. Ref.15

Domain: The N-terminal Gln-rich and Pro-rich domain has great conformational flexibility and is likely to exist in a fluctuating equilibrium of alpha-helical, random coil, and extended conformations. Ref.28

Post-translational modification: 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.

Polymorphism: The poly-Gln region of HTT is highly polymorphic (10 to 35 repeats) in the normal population and is expanded to about 36-120 repeats in Huntington disease patients. The repeat length usually increases in successive generations, but contracts also on occasion. The adjacent poly-Pro region is also polymorphic and varies between 7-12 residues. Polyglutamine expansion leads to elevated susceptibility to apopain cleavage and likely result in accelerated neuronal apoptosis.

Involvement in disease: Huntington disease (HD) [MIM:143100]: A neurodegenerative disorder characterized by involuntary movements (chorea), general motor impairment, psychiatric disorders and dementia. Onset of the disease occurs usually in the third or fourth decade of life. Onset and clinical course depend on the degree of poly-Gln repeat expansion, longer expansions resulting in earlier onset and more severe clinical manifestations. Neuropathology of Huntington disease displays a distinctive pattern with loss of neurons, especially in the caudate and putamen.Note: The disease is caused by mutations affecting the gene represented in this entry.

Sequence similarities: Belongs to the huntingtin family.Contains 5 HEAT repeats.
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 Research Articles on HTT    1. a summary of HTT protein aggregation mechanisms
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with HTT peptidePathways associated with HTT peptide
 Reference Product  PubMed Publications
 HAP1 peptide  >34 publications with HTT and HAP1
 HIP1 peptide  >23 publications with HTT and HIP1
 CREBBP peptide  >10 publications with HTT and CREBBP
 UBC peptide  >9 publications with HTT and UBC
 ZDHHC17 peptide  >7 publications with HTT and ZDHHC17
 GAPDH peptide  >6 publications with HTT and GAPDH
 SH3GL3 peptide  >4 publications with HTT and SH3GL3
 OPTN peptide  >3 publications with HTT and OPTN
 PACSIN1 peptide  >3 publications with HTT and PACSIN1
 PRPF40A peptide  >1 publications with HTT and PRPF40A
 Products by Pathway  Pathway Diagram
 Direct P53 Effectors Pathway antibodies  Direct P53 Effectors Pathway Diagram
 EGFR1 Signaling Pathway antibodies  EGFR1 Signaling Pathway Diagram
 Huntington's Disease Pathway antibodies  Huntington's Disease Pathway Diagram
 Huntington's Disease Pathway antibodies  Huntington's Disease Pathway Diagram
Diseases associated with HTT peptideOrgans/Tissues associated with HTT peptide
 Disease Name  Pubmed Publications
 Neurodegenerative Diseases Antibodies  >1340 publications with HTT and Neurodegenerative Diseases
 Huntington Disease Antibodies  >1314 publications with HTT and Huntington Disease
 Brain Diseases Antibodies  >1306 publications with HTT and Brain Diseases
 Congenital, Hereditary, and Neonatal Diseases and Abnormalities Antibodies  >1250 publications with HTT and Congenital, Hereditary, and Neonatal Diseases and Abnormalities
 Movement Disorders Antibodies  >1230 publications with HTT and Movement Disorders
 Cognition Disorders Antibodies  >1227 publications with HTT and Cognition Disorders
 Nerve Degeneration Antibodies  >166 publications with HTT and Nerve Degeneration
 Anxiety Disorders Antibodies  >157 publications with HTT and Anxiety Disorders
 Atrophy Antibodies  >101 publications with HTT and Atrophy
 Neoplasms Antibodies  >87 publications with HTT and Neoplasms
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >1227 publications with HTT and Brain
 Blood Antibodies  >291 publications with HTT and Blood
 Muscle Antibodies  >79 publications with HTT and Muscle
 Embryonic Tissue Antibodies  >71 publications with HTT and Embryonic Tissue
 Vascular Antibodies  >53 publications with HTT and Vascular
 Eye Antibodies  >38 publications with HTT and Eye
 Liver Antibodies  >37 publications with HTT and Liver
 Lung Antibodies  >31 publications with HTT and Lung
 Bone Antibodies  >29 publications with HTT and Bone
 Kidney Antibodies  >28 publications with HTT and Kidney
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