Abcam, ab175265, Anti-Ataxin 3 antibody

Size: 100µL
Rabbit Polyclonal Ataxin 3 antibody. Suitable for WB, IHC-P, ICC/IF and reacts with Human, Rat samples. Cited in 7 publications. Immunogen corresponding to Recombinant Full Length Protein corresponding to Human ATXN3.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Rat, Human,
Applications:ICC/IF, IHC-P, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Full Length Protein corresponding to Human ATXN3.P54252

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.3Preservative: 0.02% Sodium azideConstituents: PBS, 50% Glycerol (glycerin, glycerine), Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
Ataxin 3 also known as ATXN3 is a protein that functions as a deubiquitinating enzyme. It plays an important role in protein homeostasis by editing ubiquitin chains on proteins marked for degradation. This protein is approximately 42 kDa in size and is expressed in various human tissues with higher expressions in the brain and testis. Ataxin 3 contains a polyglutamine (polyQ) tract which is responsible for its aggregation in certain pathologies.
Biological function summary
At the molecular level Ataxin 3 helps in regulating protein quality control by associating with the proteasome complex. It acts as a link between ubiquitination and protein degradation pathways. The involvement of Ataxin 3 in transcriptional regulation and cytoskeletal organization further highlights its importance in cellular health. Its deubiquitinating activity implies its participation in recycling ubiquitins which are important to maintaining ubiquitin pools within the cell.
Pathways
Ataxin 3 is embedded within the ubiquitin-proteasome system which is essential for protein degradation. It links with pathways such as the protein quality control and endocytosis pathways. Ataxin 3 interacts with proteins like parkin and Ubiquitin C-terminal hydrolase L1 (UCH-L1) both of which are important for removing damaged or misfolded proteins and in the prevention of protein accumulation. These interactions show its broad role in cell maintenance and preventing toxic accumulations.
The malfunction of Ataxin 3 directly relates to Spinocerebellar Ataxia Type 3 (SCA3) also known as Machado-Joseph Disease. The elongated polyQ tract within Ataxin 3 leads to its aggregation resulting in neuronal death. This aggregation process is shared with proteins such as Ataxin 2 which also forms aggregates in certain neurological conditions indicating a pathogenic overlap with related neurodegenerative diseases. Understanding Ataxin 3’s role offers insights into therapeutic targets for these conditions.