In view of upcoming scientific trials, quantitative molecular markers accessible in peripheral blood are of important significance as prognostic or pharmacodynamic markers in genetic neurodegenerative ailments comparable to Spinocerebellar Ataxia Kind 3 (SCA3), specifically for signaling goal engagement. On this pilot examine, we centered on the quantification of ataxin-3, the protein altered in SCA3, in human peripheral blood mononuclear cells (PBMCs) acquired from preataxic and ataxic SCA3 mutation carriers in addition to wholesome controls, as a molecular marker immediately associated to SCA3 pathophysiology.
We established two completely different extremely delicate TR-FRET-based immunoassays to measure the protein ranges of both complete full-length, non-expanded and expanded, ataxin-Three or particularly polyQ-expanded ataxin-3. In PBMCs, a transparent discrimination between SCA3 mutation service and controls had been seen measuring polyQ-expanded ataxin-Three protein stage. Moreover, polyQ-expanded ataxin-Three protein ranges correlated with illness development and scientific severity as assessed by the Scale for the Evaluation and Score of Ataxia. Complete full-length ataxin-Three protein ranges had been immediately influenced by the expression ranges of the polyQ-expanded ataxin-Three protein, however weren’t correlated with scientific parameters. Evaluation of ataxin-Three ranges in fibroblasts or induced pluripotent stem cells allowed to differentiate mutation carriers from controls, thus offering proof-of-principle validation of our PBMC findings throughout cell traces.
Complete full-length or polyQ-expanded ataxin-Three protein was not detectable by TR-FRET assays in different biofluids like plasma or cerebrospinal fluid, indicating the necessity for ultra-sensitive assays for these biofluids. Standardization research revealed that tube techniques, blood sampling, and PBMC preparation could affect ataxin-Three protein ranges indicating a excessive demand for standardized protocols in biomarker research. In conclusion, the polyQ-expanded ataxin-Three protein is a promising candidate as a molecular goal engagement marker in SCA3 in future scientific trials, determinable even in-easily accessible-peripheral blood biomaterials. These outcomes, nevertheless, require validation in a bigger cohort and additional standardization of modifying situations.
Capturing the Conformational Ensemble of the Blended Folded Polyglutamine Protein Ataxin-3
Dynamics of a Protein Interplay Community Related to the Aggregation of polyQ-Expanded Ataxin-1
Rabbit Polyclonal antibody to Ataxin 3 (ataxin 3) |
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TA308320 | Origene Technologies GmbH | 100 µl | Ask for price |
Ataxin-3 Protein |
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20-abx262861 | Abbexa |
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Ataxin-3 Protein |
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abx262861-10mg | Abbexa | 10 mg | EUR 325 |
Ataxin-3 Protein |
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abx262861-25mg | Abbexa | 25 mg | EUR 6575 |
Ataxin-3 Protein |
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abx262861-5mg | Abbexa | 5 mg | EUR 225 |
Ataxin 3 protein |
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30R-1344 | Fitzgerald | 50 ug | EUR 307 |
Description: Purified recombinant Human Ataxin 3 protein |
Ataxin 3 antibody |
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22437 | SAB | 100ul | EUR 479 |
Ataxin 3 antibody |
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22437-100ul | SAB | 100ul | EUR 468 |
Ataxin 3 Antibody |
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DF6375 | Affbiotech | 200ul | EUR 420 |
Ataxin 3 Antibody |
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DF6375-100ul | Affinity Biosciences | 100ul | EUR 280 |
Ataxin 3 Antibody |
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DF6375-200ul | Affinity Biosciences | 200ul | EUR 350 |
Ataxin 3 Antibody |
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E300595 | EnoGene | 100ug/200ul | EUR 275 |
Description: Available in various conjugation types. |
Ataxin 3 antibody |
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70R-13630 | Fitzgerald | 100 ul | EUR 548 |
Description: Affinity purified Rabbit polyclonal Ataxin 3 antibody |
Ataxin 3 antibody |
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MBS9404933-01mL | MyBiosource | 0.1mL | EUR 420 |
Ataxin 3 antibody |
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MBS9404933-5x01mL | MyBiosource | 5x0.1mL | EUR 1740 |
Ataxin 3 Antibody |
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MBS853397-01mg | MyBiosource | 0.1mg | EUR 345 |
Ataxin 3 Antibody |
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MBS853397-01mLAF405L | MyBiosource | 0.1mL(AF405L) | EUR 565 |
Ataxin 3 Antibody |
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MBS853397-01mLAF405S | MyBiosource | 0.1mL(AF405S) | EUR 565 |
Ataxin 3 Antibody |
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MBS853397-01mLAF610 | MyBiosource | 0.1mL(AF610) | EUR 565 |
Ataxin 3 Antibody |
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MBS853397-01mLAF635 | MyBiosource | 0.1mL(AF635) | EUR 565 |
Ataxin 3 Antibody |
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MBS5312481-01mL | MyBiosource | 0.1mL | EUR 1065 |
Ataxin 3 Antibody |
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MBS5312481-5x01mL | MyBiosource | 5x0.1mL | EUR 4640 |
Ataxin-3 (pS256) Antibody |
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abx148457-100ug | Abbexa | 100 ug | EUR 526.8 |
Ataxin-3 Human (Recombinant) |
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22061141-1 | Glycomatrix | 2 µg | EUR 137.27 |
Recombinant Human Ataxin-3 |
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7-04462 | CHI Scientific | 2µg | Ask for price |
Recombinant Human Ataxin-3 |
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7-04463 | CHI Scientific | 10µg | Ask for price |
Recombinant Human Ataxin-3 |
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7-04464 | CHI Scientific | 1mg | Ask for price |
Ataxin-3 Human Recombinant |
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rAP-4434 | Angio Proteomie | Inquiry | Ask for price |
Recombinant Human Ataxin-3 |
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MBS143572-0002mg | MyBiosource | 0.002mg | EUR 240 |
Recombinant Human Ataxin-3 |
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MBS143572-001mg | MyBiosource | 0.01mg | EUR 310 |
Recombinant Human Ataxin-3 |
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MBS143572-1mg | MyBiosource | 1mg | EUR 5345 |
Recombinant Human Ataxin-3 |
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MBS143572-5x1mg | MyBiosource | 5x1mg | EUR 23725 |
Ataxin 3 Rabbit pAb |
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A1243-200ul | Abclonal | 200 ul | EUR 550.8 |
Ataxin 3 Rabbit pAb |
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A1243-20ul | Abclonal | 20 ul | EUR 219.6 |
Ataxin 3 Rabbit pAb |
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A12992-100ul | Abclonal | 100 ul | EUR 369.6 |
Ataxin 3 Rabbit pAb |
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A12992-200ul | Abclonal | 200 ul | EUR 550.8 |
Ataxin 3 Rabbit pAb |
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A12992-20ul | Abclonal | 20 ul | EUR 219.6 |
Ataxin 3 Rabbit pAb |
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A12992-50ul | Abclonal | 50 ul | EUR 267.6 |
Ataxin 3 Rabbit mAb |
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E2R26443 | EnoGene | 100ul | EUR 255 |
Description: Available in various conjugation types. |
Ataxin 3 Rabbit mAb |
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MBS4753194-01mL | MyBiosource | 0.1mL | EUR 450 |
Ataxin 3 Rabbit mAb |
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MBS4753194-5x01mL | MyBiosource | 5x0.1mL | EUR 1540 |
Ataxin 3 Rabbit mAb |
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MBS9467429-005mL | MyBiosource | 0.05mL | EUR 290 |
Ataxin 3 Rabbit mAb |
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MBS9467429-01mL | MyBiosource | 0.1mL | EUR 435 |
Ataxin 3 Rabbit mAb |
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MBS9467429-5x01mL | MyBiosource | 5x0.1mL | EUR 1810 |
Ataxin 3 Antibody / ATXN3 |
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F55027-0.08ML | NSJ Bioreagents | 0.08 ml | EUR 140.25 |
Description: ATXN3 was known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. |
Ataxin 3 Antibody / ATXN3 |
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F55027-0.4ML | NSJ Bioreagents | 0.4 ml | EUR 322.15 |
Description: ATXN3 was known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. |
Ataxin 3 Antibody / ATXN3 |
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R32137 | NSJ Bioreagents | 100 ug | EUR 356.15 |
Description: ATXN3 (Ataxin 3), also known as AT3, MJD GENE, MJD1, SCA3 GENE, ATX3, JOS, Spinocerebellar ataxia-3, Machado-Joseph disease protein 1, is a protein that in humans is encoded by the ATXN3 gene. ATXN3 ranges in size from 360 to 374 amino acids. Using Northern blot analysis showed that ATXN3 mRNA was ubiquitously expressed in human tissues. They detected at least 4 ATXN3 transcripts of 1.4, 1.8, 4.5, and 7.5 kb and suggested that the different mRNA species probably result from differential splicing and polyadenylation. Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by the ATXN3 gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is the cause of Machado-Joseph disease. There is an inverse correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. Ataxin-3 interacted with 2 human homologs of the yeast DNA repair protein RAD23, HHR23A (RAD23A) and HHR23B (RAD23B). Both normal and mutant ataxin-3 proteins interacted with the ubiquitin-like domain at the N terminus of the HHR23 proteins, which is a motif important for nucleotide excision repair. However, in HEK 293 cells, HHR23A was recruited to intranuclear inclusions formed by the mutant ataxin-3 through its interaction with ataxin-3. |
Ataxin-3 (ATXN3) Rabbit mAb |
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A22094 | Abclonal | 100μL | EUR 83.2 |
Description: A synthetic peptide corresponding to a sequence within amino acids 550-650 of human Neurofilament H (P12036). |
Ataxin-3 (ATXN3) Rabbit pAb |
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A1243 | Abclonal | 200μL | EUR 173.58 |
Ataxin-3 (ATXN3) Rabbit pAb |
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A12992 | Abclonal | 500μL | EUR 88.56 |
Ataxin 3 Blocking Peptide |
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MBS9625749-1mg | MyBiosource | 1mg | EUR 380 |
Ataxin 3 Blocking Peptide |
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MBS9625749-5x1mg | MyBiosource | 5x1mg | EUR 1650 |
Ataxin-10 |
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AP80722 | SAB | 1mg | EUR 2640 |
Ataxin-10 |
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AP80782 | SAB | 1mg | EUR 2640 |
Ataxin-10 |
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AP81057 | SAB | 1mg | EUR 2640 |
Ataxin-10 |
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AP81093 | SAB | 1mg | EUR 2640 |
Recombinant Ataxin 3 (ATXN3) |
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RPD250Hu01 | Cloud-Clone | 10ug | EUR 120 |
Ataxin 3 Polyclonal Antibody |
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27857-100ul | SAB | 100ul | EUR 302.4 |
Ataxin 3 Polyclonal Antibody |
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27857-50ul | SAB | 50ul | EUR 224.4 |
Ataxin 3 Polyclonal Antibody |
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A70452 | EpiGentek | 50 μl | EUR 408 |
Ataxin 3 Polyclonal Antibody |
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E-AB-60279-120uL | Elabscience Biotech | 120uL | EUR 320 |
Description: Unconjugated |
Ataxin 3 Polyclonal Antibody |
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E-AB-60279-200uL | Elabscience Biotech | 200uL | EUR 530 |
Description: Unconjugated |
Ataxin 3 Polyclonal Antibody |
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E-AB-60279-60uL | Elabscience Biotech | 60uL | EUR 200 |
Description: Unconjugated |
Ataxin 3 Polyclonal Antibody |
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E-AB-60279-each | Elabscience Biotech | each | Ask for price |
Description: Unconjugated |
Ataxin 3 Polyclonal Antibody |
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MBS9236179-01mL | MyBiosource | 0.1mL | EUR 415 |
Ataxin 3 Polyclonal Antibody |
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MBS9236179-5x01mL | MyBiosource | 5x0.1mL | EUR 1841 |
Ataxin 3 Polyclonal Antibody |
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RD80919A-120uL | Reddot Biotech | 120μL | EUR 360 |
Description: 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. |
Ataxin 3 Polyclonal Antibody |
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RD80919A-200uL | Reddot Biotech | 200μL | EUR 630 |
Description: 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. |
Ataxin 3 Polyclonal Antibody |
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RD80919A-60uL | Reddot Biotech | 60μL | EUR 204 |
Description: 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. |
Ataxin-3 Fluorescent Assay Kit |
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78044 | BPS Bioscience | 96 rxns. | EUR 765 |
Description: The Ataxin-3 Fluorescent Assay Kit is designed to measure Ataxin-3 activity for screening and profiling applications, in a homogeneous assay with no timeconsuming washing steps. |
Recombinant Rat Ataxin-3 (Atxn3) |
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MBS1243203-002mgBaculovirus | MyBiosource | 0.02mg(Baculovirus) | EUR 1230 |
Recombinant Rat Ataxin-3 (Atxn3) |
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MBS1243203-002mgEColi | MyBiosource | 0.02mg(E-Coli) | EUR 890 |
Recombinant Rat Ataxin-3 (Atxn3) |
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MBS1243203-002mgYeast | MyBiosource | 0.02mg(Yeast) | EUR 1020 |
Recombinant Rat Ataxin-3 (Atxn3) |
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MBS1243203-01mgEColi | MyBiosource | 0.1mg(E-Coli) | EUR 1070 |
Recombinant Rat Ataxin-3 (Atxn3) |
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MBS1243203-01mgYeast | MyBiosource | 0.1mg(Yeast) | EUR 1160 |
Recombinant Human Ataxin-3 (ATXN3) |
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CSB-EP002443HU | Cusabio | 11921 mg | Ask for price |
Recombinant Mouse Ataxin-3 (Atxn3) |
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MBS1457204-002mgBaculovirus | MyBiosource | 0.02mg(Baculovirus) | EUR 1230 |
Recombinant Mouse Ataxin-3 (Atxn3) |
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MBS1457204-002mgEColi | MyBiosource | 0.02mg(E-Coli) | EUR 890 |
Recombinant Mouse Ataxin-3 (Atxn3) |
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MBS1457204-002mgYeast | MyBiosource | 0.02mg(Yeast) | EUR 1020 |
Recombinant Mouse Ataxin-3 (Atxn3) |
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MBS1457204-01mgEColi | MyBiosource | 0.1mg(E-Coli) | EUR 1070 |
Recombinant Mouse Ataxin-3 (Atxn3) |
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MBS1457204-01mgYeast | MyBiosource | 0.1mg(Yeast) | EUR 1160 |
Recombinant Human Ataxin-3 (ATXN3) |
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MBS953525-002mgBaculovirus | MyBiosource | 0.02mg(Baculovirus) | EUR 1240 |
Recombinant Human Ataxin-3 (ATXN3) |
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MBS953525-002mgEColi | MyBiosource | 0.02mg(E-Coli) | EUR 900 |
Recombinant Human Ataxin-3 (ATXN3) |
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MBS953525-002mgYeast | MyBiosource | 0.02mg(Yeast) | EUR 1030 |
Recombinant Human Ataxin-3 (ATXN3) |
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MBS953525-01mgEColi | MyBiosource | 0.1mg(E-Coli) | EUR 1085 |
Recombinant Human Ataxin-3 (ATXN3) |
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MBS953525-01mgYeast | MyBiosource | 0.1mg(Yeast) | EUR 1170 |
Ataxin 3 (ATXN3) ELISA Kit |
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MBS2709514-10x96StripWells | MyBiosource | 10x96-Strip-Wells | EUR 3130 |
Ataxin 3 (ATXN3) ELISA Kit |
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MBS2709514-24StripWells | MyBiosource | 24-Strip-Wells | EUR 255 |
Ataxin 3 (ATXN3) ELISA Kit |
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MBS2709514-48StripWells | MyBiosource | 48-Strip-Wells | EUR 330 |
Ataxin 3 (ATXN3) ELISA Kit |
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MBS2709514-5x96StripWells | MyBiosource | 5x96-Strip-Wells | EUR 1630 |
Ataxin 3 (ATXN3) ELISA Kit |
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MBS2709514-96StripWells | MyBiosource | 96-Strip-Wells | EUR 420 |
Ataxin-3 (Phospho-Ser256) Antibody |
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12574 | SAB | 100ul | EUR 319 |
Ataxin-3 (Phospho-Ser256) Antibody |
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12574-100ul | SAB | 100ul | EUR 302.4 |
Ataxin-3 (Phospho-Ser256) Antibody |
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12574-50ul | SAB | 50ul | EUR 224.4 |
Recombinant Chicken Ataxin-3 (ATXN3) |
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MBS1475975-002mgBaculovirus | MyBiosource | 0.02mg(Baculovirus) | EUR 1235 |
Recombinant Chicken Ataxin-3 (ATXN3) |
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MBS1475975-002mgEColi | MyBiosource | 0.02mg(E-Coli) | EUR 900 |
Recombinant Chicken Ataxin-3 (ATXN3) |
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MBS1475975-002mgYeast | MyBiosource | 0.02mg(Yeast) | EUR 1030 |
Recombinant Chicken Ataxin-3 (ATXN3) |
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MBS1475975-01mgEColi | MyBiosource | 0.1mg(E-Coli) | EUR 1080 |
Recombinant Chicken Ataxin-3 (ATXN3) |
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MBS1475975-01mgYeast | MyBiosource | 0.1mg(Yeast) | EUR 1170 |
Recombinant Human Ataxin-3 Protein |
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MBS556290-0002mg | MyBiosource | 0.002mg | EUR 220 |
Recombinant Human Ataxin-3 Protein |
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MBS556290-001mg | MyBiosource | 0.01mg | EUR 320 |
Recombinant Human Ataxin-3 Protein |
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MBS556290-5x001mg | MyBiosource | 5x0.01mg | EUR 1300 |
Ataxin-3 (Phospho-Ser256) Antibody |
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MBS9416662-005mL | MyBiosource | 0.05mL | EUR 245 |
Ataxin-3 (Phospho-Ser256) Antibody |
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MBS9416662-01mL | MyBiosource | 0.1mL | EUR 305 |
Ataxin-3 (Phospho-Ser256) Antibody |
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MBS9416662-5x01mL | MyBiosource | 5x0.1mL | EUR 1230 |
Ataxin-3 (Phospho-Ser256) Antibody |
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A8293-100ul | Assay Biotech | 100μl | EUR 217 |
Description: Ataxin-3 (Phospho-Ser256) Rabbit Polyclonal Antibody |
Ataxin-3 (Phospho-Ser256) Antibody |
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A8293-50ul | Assay Biotech | 50μl | EUR 143.5 |
Description: Ataxin-3 (Phospho-Ser256) Rabbit Polyclonal Antibody |
Ataxin 3, 1-370aa, Human, E Coli |
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MBS203345-001mg | MyBiosource | 0.01mg | EUR 215 |
Ataxin 3, 1-370aa, Human, E Coli |
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MBS203345-005mg | MyBiosource | 0.05mg | EUR 340 |
Ataxin 3, 1-370aa, Human, E Coli |
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MBS203345-025mg | MyBiosource | 0.25mg | EUR 765 |
Ataxin 3, 1-370aa, Human, E Coli |
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MBS203345-5x025mg | MyBiosource | 5x0.25mg | EUR 3195 |
Rat Atxn3 / Ataxin-3 ELISA Kit |
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E10108r | EIAab | 96T | EUR 796 |
Rat Ataxin-3 (ATXN3) ELISA Kit |
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MBS7212062-10x96StripWells | MyBiosource | 10x96-Strip-Wells | EUR 5685 |
Rat Ataxin-3 (ATXN3) ELISA Kit |
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MBS7212062-48StripWells | MyBiosource | 48-Strip-Wells | EUR 485 |
Rat Ataxin-3 (ATXN3) ELISA Kit |
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MBS7212062-5x96StripWells | MyBiosource | 5x96-Strip-Wells | EUR 3020 |
Rat Ataxin-3 (ATXN3) ELISA Kit |
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MBS7212062-96StripWells | MyBiosource | 96-Strip-Wells | EUR 690 |
Rat Atxn3/Ataxin-3 ELISA Kit |
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MBS2903872-10x96StripWells | MyBiosource | 10x96-Strip-Wells | EUR 4080 |
Rat Atxn3/Ataxin-3 ELISA Kit |
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MBS2903872-48StripWells | MyBiosource | 48-Strip-Wells | EUR 390 |
Rat Atxn3/Ataxin-3 ELISA Kit |
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MBS2903872-5x96StripWells | MyBiosource | 5x96-Strip-Wells | EUR 2220 |
Rat Atxn3/Ataxin-3 ELISA Kit |
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MBS2903872-96StripWells | MyBiosource | 96-Strip-Wells | EUR 520 |
Goat Ataxin-3 (ATXN3) ELISA Kit |
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MBS7238168-10x96StripWells | MyBiosource | 10x96-Strip-Wells | EUR 5685 |
Goat Ataxin-3 (ATXN3) ELISA Kit |
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MBS7238168-48StripWells | MyBiosource | 48-Strip-Wells | EUR 485 |
Goat Ataxin-3 (ATXN3) ELISA Kit |
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MBS7238168-5x96StripWells | MyBiosource | 5x96-Strip-Wells | EUR 3020 |
Goat Ataxin-3 (ATXN3) ELISA Kit |
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MBS7238168-96StripWells | MyBiosource | 96-Strip-Wells | EUR 690 |
Human Ataxin-3 (ATXN3) ELISA Kit |
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AE54842HU-48Tests | Abebio | 48 Tests | EUR 325 |
Description: Human (Homo sapiens) |
Human Ataxin-3 (ATXN3) ELISA Kit |
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AE54842HU-96Tests | Abebio | 96 Tests | EUR 610 |
Description: Human (Homo sapiens) |
Human Ataxin-3 (ATXN3) ELISA Kit |
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RK10399 | Abclonal | 96T | EUR 280 |
Mouse Ataxin-3 (ATXN3) ELISA Kit |
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RK12664 | Abclonal | 96T | EUR 280 |
Human ATXN3 / Ataxin-3 ELISA Kit |
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E10108h | EIAab | 96T | EUR 736 |
Mouse Atxn3 / Ataxin-3 ELISA Kit |
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E10108m | EIAab | 96T | EUR 776 |
Human Ataxin-3 (ATXN3) ELISA Kit |
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AE54842HU-48T | Abebio | 48T | EUR 325 |
Description: Human (Homo sapiens) |
Human Ataxin-3 (ATXN3) ELISA Kit |
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AE54842HU-96T | Abebio | 96T | EUR 610 |
Description: Human (Homo sapiens) |
Sheep Ataxin-3 (ATXN3) ELISA Kit |
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MBS7237399-10x96StripWells | MyBiosource | 10x96-Strip-Wells | EUR 5685 |
Sheep Ataxin-3 (ATXN3) ELISA Kit |
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MBS7237399-48StripWells | MyBiosource | 48-Strip-Wells | EUR 485 |