Huntingtin (HTT) antibody

Antigen: Huntingtin (HTT)

Synonyms: HD, IT15, Hd, Hdh, AI256365, C430023I11Rik, hd, ZHD, HTT, HsapHD, dhtt, DmelCG9995, CG9995, Htt, HDH, SLC6A4, it15, MGC79590, htt, LOC100216476

Clonality: Monoclonal

Host: Rabbit

Reactivity: Human, Rat (Rattus)

Conjugate: Un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Flow Cytometry (FACS)

Certificates: ISO 9001:2008

Catalog no.: ABIN649903

Additional Information

Gene ID: 3064

UniProt: P42858

Description: Other names: HTT, HD, IT15, Huntingtin, Huntington disease protein

Characteristics: Rabbit Monoclonal Antibody

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

Molecular Weight: 300/260 kDA

Comments:

Background: 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).

Application Details

Application Notes: The suggested dilution is: WB: ~~ 1:1000~10000 IP: ~~ 1:30 FC: ~~ 1:40 ICC: ~~ 1:50~100 IHC: ~~ 1:250~500

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

Storage: Huntingtin (HTT) Antibody can be stored at -20°C for up to 12 months from time of receipt.

Research Area: Ubiquitin-related antibodies, Apoptosis/Necrosis, Phospho-specific antibodies, Protein Modifications, Neurology, Cell Structure

Restrictions: For Research Use only

Publications

Product: Goldberg, Nicholson, Rasper et al.: "Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract." in: Nature genetics, Vol. 13, Issue 4, pp. 442-9, 1996 (PubMed).

Mende-Mueller, Toneff, Hwang et al.: "Tissue-specific proteolysis of Huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum." in: The Journal of neuroscience : the official journal of the Society for Neuroscience, Vol. 21, Issue 6, pp. 1830-7, 2001 (PubMed).