Anti-ATXN3 Rabbit pAb

Antibody type:Primary antibody

Label:Unconjugated

Modification:Unmodification

Isotype:Rabbit IgG

Host:Rabbit

Application:WB,IF

Purify method:Affinity purified

Species:Human,Mouse

Gene Name:ATXN3

Synonyms:AT3; JOS; MJD; ATX3; MJD1; SCA3

Gene Synonyms:

Gene Full Name:ataxin 3

Gene Infomation:Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 12-44 to 52-86 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jul 2016]

Antigen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-364 of human ATXN3 (NP_004984.2).

Antigen Synonyms:Ataxin-3

Clonality:Polyclonal antibody

Source:Human

Reaction:

Form:Liquid

Tested Applications:

• Western blot (1:100 to 1:500)
• Immunofluorescence (1:50 to 1:400)
• Immunohistochemistry (1:200 to 1:500)
• Flow cytometry analysis (1:200 to 1:500)
• Enzyme-linked Immunosorbent Assay (1:100-1:5000)

Note: Users are strongly advised to determine the optimal dilution of antibody to use for their specific applications.

Clone:

Dilution:WB1:500 -1:2000; IF1:50 -1:200(Optimal dilutions should be determined by the end user)

Mole Mass:50kDa

Location:Nucleus

Concentration:

Sequence Similarity:

Gene Id:

SwissProt ID:P54252

Unigene:4287

Nucleotide Accession:

Tissue specificity:Ubiquitous.Gene expression databases

Storage:Store at -20°C. Avoid freeze / thaw cycles.

Buffer condition:PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Background:

Molar Function:Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed:12297501, PubMed:17696782, PubMed:23625928, PubMed:28445460, PubMed:16118278). 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 . Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription (PubMed:12297501). Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1 (PubMed:28445460).

Western Blot: 

Stability: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

Storage: Store at 4°C for frequent use. Stored at -20°C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.