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AB86706

Recombinant Human Ataxin 3 protein

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Recombinant Human Ataxin 3 protein is a Human Full Length protein, in the 1 to 370 aa range, expressed in Escherichia coli, with >90%, suitable for SDS-PAGE.

View Alternative Names

ATX3, MJD, MJD1, SCA3, ATXN3, Ataxin-3, Machado-Joseph disease protein 1, Spinocerebellar ataxia type 3 protein

1 Images
SDS-PAGE - Recombinant Human Ataxin 3 protein (AB86706)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human Ataxin 3 protein (AB86706)

15% SDS-PAGE showing ab86706 at approximately 42.4kDa (3μg).

Key facts

Purity

>90% SDS-PAGE

Expression system

Escherichia coli

Tags

Tag free

Applications

SDS-PAGE

applications

Biologically active

No

Accession

P54252

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 7.5 Constituents: 10% Glycerol (glycerin, glycerine), 0.316% Tris HCl, 0.29% Sodium chloride, 0.0308% (R*,R*)-1,4-Dimercaptobutan-2,3-diol

storage-buffer

Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }

Sequence info

[{"sequence":"MESIFHEKQEGSLCAQHCLNNLLQGEYFSPVELSSIAHQLDEEERMRMAEGGVTSEDYRTFLQQPSGNMDDSGFFSIQVISNALKVWGLELILFNSPEYQRLRIDPINERSFICNYKEHWFTVRKLGKQWFNLNSLLTGPELISDTYLALFLAQLQQEGYSIFVVKGDLPDCEADQLLQMIRVQQMHRPKLIGEELAQLKEQRVHKTDLERVLEANDGSGMLDEDEEDLQRALALSRQEIDMEDEEADLRRAIQLSMQGSSRNISQDMTQTSGTNLTSEELRKRREAYFEKQQQKQQQQQQQQQQQQQQQQQQQGDLSGQSSHPCERPATSSGALGSDLGDAMSEEDMLQAAVTMSLETVRNDLKTEGKK","proteinLength":"Full Length","predictedMolecularWeight":"42.4 kDa","actualMolecularWeight":null,"aminoAcidEnd":370,"aminoAcidStart":1,"nature":"Recombinant","expressionSystem":"Escherichia coli","accessionNumber":"P54252","tags":[]}]

Properties and storage information

Shipped at conditions
Blue Ice
Appropriate short-term storage conditions
-20°C
Appropriate long-term storage conditions
-20°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
False

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.

Specifications

Form

Liquid

Additional notes

ab86706 is purified using conventional chromatography techniques.Endotoxin level: < 1.0 EU per 1 μg of protein (determined by LAL method).

General info

Function

Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed : 12297501, PubMed : 16118278, PubMed : 17696782, PubMed : 23625928, PubMed : 28445460, PubMed : 33157014). Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins (PubMed : 17696782). Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP : recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension (By similarity). Interacts with key regulators of transcription and represses transcription : acts as a histone-binding protein that regulates transcription (PubMed : 12297501). Acts as a negative regulator of mTORC1 signaling in response to amino acid deprivation by mediating deubiquitination of RHEB, thereby promoting RHEB inactivation by the TSC-TBC complex (PubMed : 33157014). Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1 (PubMed : 28445460).

Post-translational modifications

Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity (PubMed:23696636).

Subcellular localisation

Nucleus matrix

Product protocols

Target data

Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed : 12297501, PubMed : 16118278, PubMed : 17696782, PubMed : 23625928, PubMed : 28445460, PubMed : 33157014). Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins (PubMed : 17696782). Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP : recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension (By similarity). Interacts with key regulators of transcription and represses transcription : acts as a histone-binding protein that regulates transcription (PubMed : 12297501). Acts as a negative regulator of mTORC1 signaling in response to amino acid deprivation by mediating deubiquitination of RHEB, thereby promoting RHEB inactivation by the TSC-TBC complex (PubMed : 33157014). Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1 (PubMed : 28445460).
See full target information ATXN3

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