Huntingtin antibody (AA 1247-1646)

Catalog No. ABIN5540397

The Mouse Monoclonal anti-Huntingtin antibody has been validated for IHC (p). It is suitable to detect Huntingtin in samples from Human and Mouse.

Quick Overview for Huntingtin antibody (AA 1247-1646) (ABIN5540397)

Target: Huntingtin (HTT)

Reactivity: Human, Mouse

Host: Mouse

Clonality: Monoclonal

Conjugate: This Huntingtin antibody is un-conjugated

Application: Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Clone: 3HU-4E6

Product Details anti-Huntingtin Antibody

Binding Specificity: AA 1247-1646

Specificity: This antibody detects Huntingtin at aa 1247-1646.

Purification: Affinity Chromatography

Immunogen: Recombinant Huntingtin fragment amino acids 1247 to 1646

Isotype: IgG2b

Application Details

Application Notes: Immunohistochemistry on Paraffin Sections: 2 μg/mL (1/100). Microwave pretreatment in citrate buffer is recommended for antigen retrieval. Suggested Positive Control: Human hypothalamus.

Restrictions: For Research Use only

Handling

Format: Lyophilized

Buffer: PBS, pH 7.2 Preservatives: 0.01 % Kathon Stabilizers: 5 mg/mL BSA

Storage: 4 °C,-20 °C

Storage Comment: Store lyophilized at 2-8°C for 6 months or at -20°C long term. After reconstitution store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C long term. Avoid repeated freezing and thawing. Shelf life: one year from despatch.

Expiry Date: 12 months

Target Details for Huntingtin

Target: Huntingtin (HTT)

Alternative 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 trans lates 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 p olyglutamine 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] Antigen distribution: Nucleus but also cytoplasmic expression in most tissues. Highest levels in neurons and paneth cells in gastrointestinal tract. Distinct granular expression pattern in several glandular epithelia.

UniProt: P42858

Pathways: PI3K-Akt Signaling, Hormone Transport, Transition Metal Ion Homeostasis, Tube Formation, Protein targeting to Nucleus, Dicarboxylic Acid Transport