Huntingtin (HTT) antibody

Details for Product No. ABIN649903
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Antigen
Synonyms HTT, hd, ZHD, CG9995, Dmel\\CG9995, HD, Hsap\\HD, Htt, dHtt, dhtt, SLC6A4, huntington, it15, htt, IT15, AI256365, C430023I11Rik, Hd, Hdh
Reactivity
Human, Rat (Rattus)
(91), (40), (37), (13), (13), (12), (12), (3), (3)
Host
Rabbit
(52), (42), (2), (1)
Clonality
Monoclonal
Conjugate
Un-conjugated
(1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Western Blotting (WB), Immunohistochemistry (IHC)
(72), (48), (29), (20), (19), (15), (10), (8), (7), (5), (4), (2), (1)
Pubmed 2 references available
Quantity 100 μL
Options
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Catalog No. ABIN649903
450.00 $
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Specificity A synthetic peptide corresponding to residues specific to the apopain cleavage site of human huntingtin protein was used as an immunogen
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).
Synonyms: HTT, HD, IT15, Huntingtin, Huntington disease protein
Molecular Weight 300/260 kDA
Gene ID 3064
UniProt P42858
Research Area Ubiquitin-related antibodies, Apoptosis/Necrosis, Phospho-specific antibodies, Protein Modifications, Neurology, Cell Structure
Application Notes IHC: = 1:250-500, WB: = 1:1000-10000
Comment

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).

Restrictions For Research Use only
Format Liquid
Buffer 50 mM Tris-Glycine (pH 7.4), 0.15 M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage -20 °C
Storage Comment Huntingtin (HTT) Antibody can be stored at -20°C for up to 12 months from time of receipt.
Expiry Date 12 months
Background publications 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).

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