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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abx262861-10mg | Abbexa | 10 mg | EUR 325 |
Ataxin-3 Protein |
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20-abx262861 | Abbexa |
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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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70R-13630 | Fitzgerald | 100 ul | EUR 548 |
Description: Affinity purified Rabbit polyclonal Ataxin 3 antibody |
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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DF6375 | Affbiotech | 200ul | EUR 420 |
Ataxin 3 Antibody |
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DF6375-100ul | Affinity Biosciences | 100ul | EUR 168 |
Description: WB,IHC,ELISA(peptide) |
Ataxin 3 Antibody |
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DF6375-200ul | Affinity Biosciences | 200ul | EUR 210 |
Description: WB,IHC,ELISA(peptide) |
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 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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MBS9404933-01mL | MyBiosource | 0.1mL | EUR 420 |
Ataxin 3 antibody |
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MBS9404933-5x01mL | MyBiosource | 5x0.1mL | EUR 1740 |
Ataxin-3 (pS256) Antibody |
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abx148457-100ug | Abbexa | 100 ug | EUR 526.8 |
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 |
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 Human Recombinant |
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rAP-4434 | Angio Proteomie | Inquiry | Ask for price |
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 pAb |
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E45R19058N | EnoGene | 50 ul | EUR 297.75 |
Ataxin 3 Rabbit pAb |
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E45R19588N | EnoGene | 50 ul | EUR 297.75 |
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 (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 (ATXN3) Rabbit mAb |
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A22094 | Abclonal | 100μL | EUR 343.62 |
Description: A synthetic peptide corresponding to a sequence within amino acids 550-650 of human Neurofilament H (P12036). |
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 |
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 |
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 |
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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 monoclonal antibody |
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MB11923 | Bioworld Biotech | 50ul | EUR 398 |
Description: 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40% Glycerol, 0.01% Sodium azide and 0.05% BSA |
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 Human Ataxin-3 (ATXN3) |
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CSB-EP002443HU-100ug | Cusabio | 100ug | Ask for price |
Description: 1-364aa |
Recombinant Human Ataxin-3 (ATXN3) |
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CSB-EP002443HU-1mg | Cusabio | 1mg | Ask for price |
Description: 1-364aa |
Recombinant Human Ataxin-3 (ATXN3) |
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CSB-EP002443HU-20ug | Cusabio | 20ug | Ask for price |
Description: 1-364aa |
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 |
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 (ATXN3) polyclonal antibody |
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BS74860 | Bioworld Biotech | 50ul | EUR 358 |
Description: 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2 |
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, 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 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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E10108r | EIAab | 96T | EUR 796 |
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 |
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 |
Sheep Ataxin-3 (ATXN3) ELISA Kit |
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MBS7237399-5x96StripWells | MyBiosource | 5x96-Strip-Wells | EUR 3020 |
Sheep Ataxin-3 (ATXN3) ELISA Kit |
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MBS7237399-96StripWells | MyBiosource | 96-Strip-Wells | EUR 690 |
Mouse Ataxin-3 (ATXN3) ELISA Kit |
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MBS7248783-10x96StripWells | MyBiosource | 10x96-Strip-Wells | EUR 5685 |
Mouse Ataxin-3 (ATXN3) ELISA Kit |
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MBS7248783-48StripWells | MyBiosource | 48-Strip-Wells | EUR 485 |
Mouse Ataxin-3 (ATXN3) ELISA Kit |
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MBS7248783-5x96StripWells | MyBiosource | 5x96-Strip-Wells | EUR 3020 |