Huntingtin Products

Description

Huntingtin protein (Htt) is a 348 kDa protein product of Huntington's disease (HD, IT15) gene. Huntington's 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 Huntington's 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).

Bioinformatics

Entrez	15194 Mouse 29424 Rat 3064 Human
Uniprot	AAB38240 Human NP_002102 Human P42858 Human P51111 Rat
Product By Gene ID	3064
Alternate Names	HD protein huntingtin (Huntington disease) huntingtin IT15HDHuntington disease protein

Research Areas for Huntingtin

Find related products by research area and learn more about each of the different research areas below.

Apoptosis
Autophagy
Cytoskeleton Markers
Neurodegeneration
Neuronal Cell Markers
Neuroscience
Phospho-Specific

Related Huntingtin Blog Posts

Check out the latest blog posts on Huntingtin.

Mechanisms of Neurodegeneration: Protein aggregation and failure of autophagy By Michalina Hanzel, PhDIn a series of three blog posts I will briefly explore the major cellular mechanisms responsible for many neurodegenerative disorders. The first, and perhaps the most apparent, is the accumulat...    Read more.

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