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Huntingtin (HTT) Antibody

Catalog # Availability Size / Price Inquiry
APR12411G 100 μl / $545

Huntingtin (HTT) Antibody

Brand

Leading Biology

Catalog Number

APR12411G

Product Type

Monoclonal Antibodies

Field of Research

Product Overview

We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format. We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team. This product is a high quality Huntingtin (HTT) antibody.

Molecular Weight

347603 Da

Cellular Localization

Antigen 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

Host

Rabbit

Species Reactivity

Human, Mouse, Rat

Target

A synthetic peptide corresponding to residues specific to the apopain cleavage site of human huntingtin protein was used as an immunogen

Clone

EP867Y

Symbol

HD, IT15

GeneID

UniProt ID

Function

May play a role in microtubule-mediated transport or vesicle function.

Summary

Huntingtin protein (Htt) is a 348 kDa protein product of Huntingtons disease (HD, IT15) gene. Huntingtons disease is a neurodegenerative disorder caused by a mutation on the HD gene, producing a polyglutamin (polyQ) expansion on the N-terminus of Htt. The mutant Htt leads to cytotoxicity in the striatal neuron (1). This cytotoxicity is modulated by proteolytic cleavage with caspases and calpains, producing N-terminal polyQ fragments. A phosphorylation of Htt may also regulate the cleavage and cytotoxicity of mutant Htt (2). The wild-type Htt up-regulates transcription of brain-derived neurotrophic factor (BDNF), a pro-survival factor for striatal neurons. This suggests restoring wild-type Htt level and increasing BDNF expression level can be therapeutic treatment for Huntingtons disease (3). Huntingtin is also cleaved by apopain, a cysteine protease product of death-gene for human, suggesting HD might be a disorder of inappropriate apoptosis (4).

Form

50 mM Tris-Glycine (pH 7.4), 0.15 M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.

Storage & Stability

Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

Applications

WB, IHC

Dilution

WB~~1:1000~10000 IHC~~1:250~500

Synonyms

Huntingtin, Huntington disease protein, HD protein, HTT, HD, IT15

Images

A. Western blot analysis on SH-SY-5Y cell lysate using anti-huntingtin RabMAb (Cat. APR12411G), dilution 1:10,000.

B. Immunohistochemical analysis of paraffin-embedded human brain tissue using RabMAb. (Cat. APR12411G).

Specification

Quantity

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