antibody

Rabbit Polyclonal to Human HTT / Huntingtin

MBS240583

0.05 mg

495 USD

polyclonal

rabbit

nonconjugated

western blot, ELISA, immunohistochemistry, enzyme immunoassay

Antibody

Rabbit Polyclonal to Human HTT / Huntingtin

HTT / Huntingtin

Anti-HTT / Huntingtin Antibody (phospho-Ser421) IHC-plus; HTT; Huntingtin; Huntington disease protein; IT15; HD; HD protein; Human HTT; Huntingtin

huntingtin; Huntingtin; huntingtin; huntington disease protein; huntingtin; Huntington disease protein; HD protein

HTT

HTT; HTT; HD; IT15; HD; IT15; HD protein

HD_HUMAN

Polyclonal

Rabbit

Human

3144

Amino acids 416 to 424 of Human huntingtin protein phosphorylated at Ser421

Immunoaffinity Purified

0.02 M potassium phosphate, 0.15 M sodium chloride, pH 7.2, 0.01% sodium azide.

0.8 mg/ml

Long term: -20 degree C; Short term: +4 degree C. Avoid repeat freeze-thaw cycles.

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

ELISA (1:10000 - 1:40000), IHC-P (10 - 20 ug/ml), WB (1:500 - 1:3000). Usage: Immunohistochemistry: was validated for use in immunohistochemistry on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with the primary antibo.

Target Species: Human. Immunogen Description: Synthetic peptide from human HTT / Huntingtin.

Immunogen Type: Synthetic peptide. Immunogen: HTT / Huntingtin antibody was raised against synthetic peptide from human HTT / Huntingtin. Antigen Modification: pSer421

Family: Hungtintin

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.

90903231

NP_002102.4

NM_002111.7

P42858

347,603 Da

Direct P53 Effectors Pathway (137939); EGFR1 Signaling Pathway (198782); Huntington's Disease Pathway (83100); Huntington's Disease Pathway (512)

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]

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 adult 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; beta-tubulin binding; dynein intermediate chain binding; diazepam binding; transcription factor binding. Biological Process: ER to Golgi vesicle-mediated transport; citrulline metabolic process; paraxial mesoderm formation; regulation of protein phosphatase type 2A activity; regulation of synaptic plasticity; locomotory behavior; determination of adult life span; endosome transport; anterior/posterior pattern formation; L-glutamate import; regulation of mitochondrial membrane potential; establishment of mitotic spindle orientation; protein import into nucleus; quinolinate biosynthetic process; organ development; 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; neuron development; hormone metabolic process; spermatogenesis; regulation of mitochondrial membrane permeability; response to calcium ion; neural plate formation; urea cycle. Disease: Huntington Disease

0.05 mg

495 USD