ATXN3 sirna

ATXN3 siRNA (Mouse)

Cat. Num. AAA8217841

• Gene Names: Atxn3; Mjd; ATX3; MJD1; Sca3; AI463012; AI647473; ataxin-3; 2210008M02Rik
• Reactivity: Mouse
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: ATXN3; ATXN3 siRNA (Mouse); MJD; Ataxin-3; Machado-Joseph disease protein 1 homolog; ATXN3 sirna

• Host: Synthetic
• Reactivity: Mouse
• Specificity: ATXN3 siRNA (Mouse) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 355

• Applicable Applications for ATXN3 sirna: RNA Interference (RNAi)
• Quality Control: Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
• Directions for Use: We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
• Components: We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of mouse ATXN3 gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
• Preparation and Storage: Shipped at 4 degree C. Store at -20 degree C for one year.

Related Product Information for ATXN3 sirna

siRNA to inhibit ATXN3 expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 268837077
• NCBI GeneID: 110616
• NCBI Accession #: NP_083981.2
• NCBI GenBank Nucleotide #: NM_029705.3
• UniProt Accession #: Q9CVD2
• Molecular Weight: 40,533 Da
• NCBI Official Full Name: ataxin-3 isoform 1
• NCBI Official Synonym Full Names: ataxin 3
• NCBI Official Symbol: Atxn3
• NCBI Official Synonym Symbols: Mjd; ATX3; MJD1; Sca3; AI463012; AI647473; ataxin-3; 2210008M02Rik
• NCBI Protein Information: ataxin-3
• UniProt Protein Name: Ataxin-3
• Protein Family: Ataxin
• UniProt Gene Name: Atxn3
• UniProt Synonym Gene Names: Mjd
• UniProt Entry Name: ATX3_MOUSE

Uniprot Description

ataxin-3: Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. 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. In response to misfolded substrate ubiquitination, mediates deubiquitination of monoubiquitinated STUB1/CHIP. Interacts with key regulators of transcription and represses transcription: acts as a histone- binding protein that regulates transcription. Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3); also known as Machado-Joseph disease (MJD). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Transcription regulation; Protease; EC 3.4.19.12; Ubiquitin-specific protease; DNA repair, damage

Cellular Component: mitochondrial matrix; mitochondrial membrane; cytoplasm; cytosol; nucleus; nuclear inclusion body

Molecular Function: peptidase activity; identical protein binding; omega peptidase activity; protein binding; hydrolase activity; ubiquitin protein ligase binding; ubiquitin-specific protease activity; histone deacetylase activity; ATPase binding; cysteine-type peptidase activity

Biological Process: ubiquitin-dependent protein catabolic process; proteasomal ubiquitin-dependent protein catabolic process; transcription, DNA-dependent; regulation of transcription, DNA-dependent; misfolded or incompletely synthesized protein catabolic process; intermediate filament cytoskeleton organization and biogenesis; microtubule cytoskeleton organization and biogenesis; proteolysis; actin cytoskeleton organization and biogenesis