HTT / Huntingtin, Monoclonal Antibody

Anti-HTT / Huntingtin Antibody (clone 3F1) IHC-plus; HTT; Huntingtin; Huntington disease protein; IT15; HD; HD protein; Human HTT; Huntingtin

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG2a,k

3F1

Mouse

Human Huntingtin

Protein A Purified

PBS, pH 7.2

0.48 mg/ml (lot specific)

Short term 4 degree C, long term aliquot and store at -20 degree C, avoid freeze thaw cycles.

Small volumes of anti-HTT antibody 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.

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.

Family: Hungtintin

Immunohistochemistry (IHC - Paraffin), Western Blot (WB), ELISA (EIA)

ELISA, IHC-P (5 ug/ml), WB
Usage: Immunohistochemistry: Formalin-fixed paraffin-embedded sections. Sandwich ELISA: Recombinant protein. Western Blot using cell lysates and the recombinant protein used as the immunogen.

Anti-HTT / Huntingtin antibody IHC of human brain, cerebellum. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.

HD monoclonal antibody clone 3F1. Western blot of HD expression in U-2 OS.

Detection limit for recombinant GST tagged HD is approximately 0.3 ng/ml as a capture antibody.

347,603 Da

huntingtin

huntingtin; huntington disease protein

Huntingtin

HD; IT15; HD protein  [Similar Products]

HD_HUMAN

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 ***** tissues. The larger transcript is approximately 13.7 kb and is expressed predominantly in ***** 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]

Huntingtin: may play a role in microtubule-mediated transport or vesicle function. 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. Defects are the cause of 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. 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 isoforms displaying different relative abundance in various fetal and ***** tissues.

Protein type: Cytoskeletal

Chromosomal Location of Human Ortholog: 4p16.3

Cellular Component: Golgi apparatus; cytoplasmic vesicle membrane; protein complex; mitochondrion; endoplasmic reticulum; dendrite; autophagic vacuole; inclusion body; cytosol; nucleoplasm; axon; cytoplasm; late endosome; nucleus

Molecular Function: identical protein binding; protein binding; p53 binding; dynein intermediate chain binding; beta-tubulin binding; diazepam binding; transcription factor binding

Biological Process: ER to Golgi vesicle-mediated transport; paraxial mesoderm formation; citrulline metabolic process; regulation of protein phosphatase type 2A activity; regulation of synaptic plasticity; locomotory behavior; determination of ***** life span; endosome transport; anterior/posterior pattern formation; L-glutamate import; establishment of mitotic spindle orientation; regulation of mitochondrial membrane potential; protein import into nucleus; organ development; quinolinate biosynthetic process; retrograde vesicle-mediated transport, Golgi to ER; vesicle transport along microtubule; visual learning; negative regulation of neuron apoptosis; Golgi organization and biogenesis; grooming behavior; endoplasmic reticulum organization and biogenesis; positive regulation of inositol-1,4,5-triphosphate receptor activity; striatum development; axon cargo transport; cell aging; olfactory lobe development; social behavior; lactate biosynthetic process from pyruvate; iron ion homeostasis; neuron apoptosis; insulin secretion; dopamine receptor signaling pathway; hormone metabolic process; neuron development; spermatogenesis; regulation of mitochondrial membrane permeability; response to calcium ion; neural plate formation; urea cycle

Disease: Huntington Disease

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