3-Methylglutaconic (3MGC) aciduria is a typical phenotypic function of a rising quantity of inborn errors of metabolism. “Primary” 3MGC aciduria is attributable to deficiencies in leucine pathway enzymes whereas “secondary” 3MGC aciduria outcomes from inborn errors of metabolism that impression mitochondrial vitality manufacturing. The metabolic precursor of 3MGC acid is trans-3MGC CoA, an intermediate within the leucine catabolism pathway. Gas chromatography-mass spectrometry (GC-MS) evaluation of commercially out there trans-3MGC acid yielded a combination of cis and trans isomers whereas 1H-NMR spectroscopy of trans-3MGC acid at 25°C supplied no proof for the cis isomer.
When trans-3MGC acid was incubated beneath circumstances used for pattern derivatization previous to GC-MS (however with no trimethylsilane added), 1H-NMR spectroscopy supplied proof of trans to cis isomerization. Incubation of trans-3MGC acid at 37°C resulted in time-dependent isomerization to cis-3MGC acid. Cis-3MGC acid behaved in the same method besides that, beneath equivalent incubation circumstances, much less isomerization occurred. In settlement with these experimental outcomes, molecular modeling research supplied proof that the vitality minimized construction of cis-3MGC acid is four kJ/mol extra secure than that for trans-3MGC acid.
The knowledge introduced are according to the incidence of each diastereomers in urine samples of topics with 3MGC aciduria. Quantification of iron is a crucial step to evaluate the iron burden in sufferers affected by iron overload illnesses, in addition to super worth in understanding the underlying function of iron within the pathophysiology of these illnesses. Current iron willpower of complete or labile iron, requires in depth pattern dealing with and specialised devices, while being time consuming and laborious. Moreover, there’s minimal to no overlap between complete iron and labile iron quantification methodologies-i.e. requiring totally separate protocols, strategies and devices. Herein, we report a unified-ferene (u-ferene) assay that allows a 2-in-1 quantification of each labile and complete iron from the identical preparation of a organic specimen.
MANTI: Automated Annotation of Protein N-Termini for Rapid Interpretation of N-Terminome Data Sets
Site-specific proteolytic processing is a crucial, irreversible post-translational protein modification with implications in lots of illnesses. Enrichment of protein N-terminal peptides adopted by mass spectrometry-based identification and quantification allows proteome-wide characterization of proteolytic processes and protease substrates however is challenged by the shortage of particular annotation instruments. A typical downside is, for instance, ambiguous matches of recognized peptides to a number of protein entries within the databases used for identification.
We developed MaxQuant Advanced N-termini Interpreter (MANTI), a standalone Perl software program with an optionally available graphical consumer interface that validates and annotates N-terminal peptides recognized by database searches with the favored MaxQuant software program bundle by integrating data from a number of knowledge sources. MANTI makes use of numerous annotation data in a multistep determination course of to assign a conservative most well-liked protein entry for every N-terminal peptide, enabling automated classification in line with the doubtless origin and determines important adjustments in N-terminal peptide abundance. Auxiliary R scripts included within the software program bundle summarize and visualize key facets of the information.
To showcase the utility of MANTI, we generated two large-scale TAILS N-terminome knowledge units from two completely different animal fashions of chemically and genetically induced kidney illness, puromycin adenonucleoside-treated rats (PAN), and heterozygous Wilms Tumor protein 1 mice (WT1). MANTI enabled fast validation and autonomous annotation of >10 000 recognized terminal peptides, revealing novel proteolytic proteoforms in 905 and 644 proteins, respectively. Quantitative evaluation indicated that proteolytic actions with related sequence specificity are concerned within the pathogenesis of kidney harm and proteinuria in each fashions, whereas coagulation processes and complement activation have been particularly induced after chemical harm.
ALS/FTD-causing mutation in cyclin F causes the dysregulation of SFPQ
Previously, we recognized missense mutations in CCNF which can be causative of familial and sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Hallmark options of these illnesses embody the build-up of insoluble protein aggregates in addition to the mislocalisation of proteins reminiscent of TDP-43. In latest years, the dysregulation of SFPQ has additionally emerged as a pathological hallmark of ALS/FTD. CCNF encodes for the protein cyclin F, a substrate recognition part of an E3-ubiquitin ligase. We have beforehand proven that ALS/FTD-linked mutations in CCNF trigger disruptions to general protein homeostasis that results in a build-up of Ok48-linked ubiquitylated proteins in addition to defects in autophagic equipment.
To examine additional processes that could be affected by cyclin F, we used a protein-proximity ligation methodology, often known as BioID, customary immunoprecipitations and mass spectrometry (MS) to establish novel interplay companions of cyclin F and infer additional course of that could be affected by the ALS/FTD-causing mutation. Results display that cyclin F intently associates with proteins concerned with RNA metabolism in addition to a quantity of RNA binding proteins beforehand linked to ALS/FTD, together with splicing issue proline and glutamine wealthy (SFPQ).
RPA Interacting Protein Antibody |
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| 4015-01mg | ProSci | 0.1 mg | EUR 436.42 |
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Description: RPA Interacting Protein Antibody: Replication protein A (RPA) is a single-stranded-DNA binding protein involved in numerous eukaryotic DNA processes including replication, repair and recombination. RPA interacting protein (RPA IP) has been identified as an adapter protein that is involved in RPA nuclear import instead of the prototypical importin proteins that normally mediate nuclear import. Multiple isoforms of RPA IP are known to exist, with the longest isoform localized to the cytoplasm. Isoform 2 is sumoylated and is located in the PML nuclear body within the nucleus. It has been suggested that this isoform mediates the localization of the RPA complex into the PML nuclear body, thereby participating in RPA function in DNA metabolism. |
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RPA Interacting Protein |
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| 20-abx262685 | Abbexa |
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RPA-Interacting Protein (RPAIN) Antibody |
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| abx028958-400ul | Abbexa | 400 ul | EUR 523 |
RPA-Interacting Protein (RPAIN) Antibody |
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| abx028958-80l | Abbexa | 80 µl | EUR 286 |
RPA-Interacting Protein (RPAIN) Antibody |
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| abx237394-100ug | Abbexa | 100 ug | EUR 551 |
RPA Interacting Protein Peptide |
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| 4013P | ProSci | 0.05 mg | EUR 164.75 |
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Description: (CT) RPA Interacting Protein peptide |
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RPA Interacting Protein Peptide |
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| 4015P | ProSci | 0.05 mg | EUR 164.75 |
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Description: (NT) RPA Interacting Protein peptide |
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RPAIN RPA Interacting Protein Human Recombinant Protein |
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| PROTQ86UA6 | BosterBio | Regular: 10ug | EUR 317 |
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Description: RPAIN Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 129 amino acids (1-106 a.a.) and having a molecular mass of 14.7kDa.RPAIN is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques. |
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Human RPA-interacting protein (RPAIN) ELISA Kit |
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| abx382885-96tests | Abbexa | 96 tests | EUR 911 |
Mouse RPA-interacting protein (RPAIN) ELISA Kit |
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| abx390479-96tests | Abbexa | 96 tests | EUR 911 |
Rat RPA-interacting protein (RPAIN) ELISA Kit |
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| abx391921-96tests | Abbexa | 96 tests | EUR 911 |
Mouse RPA- interacting protein, Rpain ELISA KIT |
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| ELI-42588m | Lifescience Market | 96 Tests | EUR 865 |
Human RPA- interacting protein, RPAIN ELISA KIT |
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| ELI-22025h | Lifescience Market | 96 Tests | EUR 824 |
Rpain ELISA Kit| Mouse RPA-interacting protein ELISA Kit |
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| EF016121 | Lifescience Market | 96 Tests | EUR 689 |
Rpain ELISA Kit| Rat RPA-interacting protein ELISA Kit |
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| EF019281 | Lifescience Market | 96 Tests | EUR 689 |
RPA p32 (phospho Ser33) Polyclonal Antibody |
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| ES1439-50ul | ELK Biotech | 50ul | EUR 207 |
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Description: A Rabbit Polyclonal antibody against RPA p32 (phospho Ser33) from Human/Mouse/Rat. This antibody is tested and validated for WB, ELISA, WB, ELISA |
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RPA p32 (phospho Ser33) Polyclonal Antibody |
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| ES1439-100ul | ELK Biotech | 100ul | EUR 279 |
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Description: A Rabbit Polyclonal antibody against RPA p32 (phospho Ser33) from Human/Mouse/Rat. This antibody is tested and validated for WB, ELISA, WB, ELISA |
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RPA p32 (phospho Ser33) Polyclonal Antibody |
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| ABP50440-003ml | Abbkine | 0.03ml | EUR 158 |
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Description: A polyclonal antibody for detection of RPA p32 phospho Ser33) from Human, Mouse, Rat. This RPA p32 phospho Ser33) antibody is for WB , ELISA. It is affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogenand is unconjugated. The antibody is produced in rabbit by using as an immunogen synthesized peptide derived from human RPA p32 around the phosphorylation site of S33 |
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RPA p32 (phospho Ser33) Polyclonal Antibody |
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| ABP50440-01ml | Abbkine | 0.1ml | EUR 289 |
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Description: A polyclonal antibody for detection of RPA p32 phospho Ser33) from Human, Mouse, Rat. This RPA p32 phospho Ser33) antibody is for WB , ELISA. It is affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogenand is unconjugated. The antibody is produced in rabbit by using as an immunogen synthesized peptide derived from human RPA p32 around the phosphorylation site of S33 |
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RPA p32 (phospho Ser33) Polyclonal Antibody |
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| ABP50440-02ml | Abbkine | 0.2ml | EUR 414 |
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Description: A polyclonal antibody for detection of RPA p32 phospho Ser33) from Human, Mouse, Rat. This RPA p32 phospho Ser33) antibody is for WB , ELISA. It is affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogenand is unconjugated. The antibody is produced in rabbit by using as an immunogen synthesized peptide derived from human RPA p32 around the phosphorylation site of S33 |
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RPA 70 kDa subunit antibody |
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| 22833-100ul | SAB | 100ul | EUR 390 |
RPA 14 kDa subunit antibody |
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| 22947-100ul | SAB | 100ul | EUR 390 |
RPA 32 kDa subunit antibody |
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| 23069-100ul | SAB | 100ul | EUR 390 |
Anti-Phospho-RPA p32 (S33) Antibody |
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| P02067 | BosterBio | 100ul | EUR 397 |
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Description: Rabbit Polyclonal Antibody for Phospho-RPA p32 (S33) Antibody (RPA2) detection. Tested with WB in Human, Mouse, Rat. |
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Anti-Phospho-RPA p32 (S33) antibody |
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| STJ90636 | St John's Laboratory | 200 µl | EUR 197 |
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Description: Rabbit polyclonal to Phospho-RPA p32 (S33). |
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Polyclonal HSC70 Interacting Protein HIP (ST13) Antibody |
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| APR16725G | Leading Biology | 0.1ml | EUR 484 |
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Description: A polyclonal antibody raised in Rabbit that recognizes and binds to Human HSC70 Interacting Protein HIP (ST13) . This antibody is tested and proven to work in the following applications: |
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Toll Interacting Protein (TOLLIP) Polyclonal Antibody (Human) |
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| 4-PAC803Hu01 | Cloud-Clone |
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Description: A Rabbit polyclonal antibody against Human Toll Interacting Protein (TOLLIP) |
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PAK-interacting Protein 1 (PAK1IP1) polyclonal antibody |
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| ABP-PAB-10259 | Allele Biotech | 100 ug | Ask for price |
Huntingtin-associated protein interacting protein (HAPIP) polyclonal antibody |
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| ABP-PAB-10621 | Allele Biotech | 100 ug | Ask for price |
Rabbit Cytohesin Interacting Protein ELISA kit |
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| E04C0575-192T | BlueGene | 192 tests | EUR 1270 |
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Description: A competitive ELISA for quantitative measurement of Rabbit Cytohesin Interacting Protein in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species. |
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Rabbit Cytohesin Interacting Protein ELISA kit |
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| E04C0575-48 | BlueGene | 1 plate of 48 wells | EUR 520 |
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Description: A competitive ELISA for quantitative measurement of Rabbit Cytohesin Interacting Protein in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species. |
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Rabbit Cytohesin Interacting Protein ELISA kit |
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| E04C0575-96 | BlueGene | 1 plate of 96 wells | EUR 685 |
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Description: A competitive ELISA for quantitative measurement of Rabbit Cytohesin Interacting Protein in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species. |
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Rabbit Hedgehog Interacting Protein ELISA kit |
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| E04H0058-192T | BlueGene | 192 tests | EUR 1270 |
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Description: A competitive ELISA for quantitative measurement of Rabbit Hedgehog Interacting Protein in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species. |
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Rabbit Hedgehog Interacting Protein ELISA kit |
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| E04H0058-48 | BlueGene | 1 plate of 48 wells | EUR 520 |
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Description: A competitive ELISA for quantitative measurement of Rabbit Hedgehog Interacting Protein in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species. |
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Rabbit Hedgehog Interacting Protein ELISA kit |
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| E04H0058-96 | BlueGene | 1 plate of 96 wells | EUR 685 |
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Description: A competitive ELISA for quantitative measurement of Rabbit Hedgehog Interacting Protein in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species. |
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Rat Pancreatic Amylase Antibody (Clone RPA-B5) |
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| 3102-100 | Biovision | EUR 338 | |
CD4 FITC Monoclonal Antibody (Clone RPA-T4) |
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| 6953-100 | Biovision | EUR 251 | |
CD4 FITC Monoclonal Antibody (Clone RPA-T4) |
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| 6953-25 | Biovision | EUR 131 | |
Anti-CD2 Antibody [RPA-2.10], APC-100Tests |
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| QAB1-APC-100Tests | EnQuireBio | 100Tests | EUR 369 |
Anti-CD2 Antibody [RPA-2.10], APC-25Tests |
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| QAB1-APC-25Tests | EnQuireBio | 25Tests | EUR 200 |
Anti-CD2 Antibody [RPA-2.10], APC-500Tests |
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| QAB1-APC-500Tests | EnQuireBio | 500Tests | EUR 1367 |
Anti-CD2 Antibody [RPA-2.10], FITC-100Tests |
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| QAB1-F-100Tests | EnQuireBio | 100Tests | EUR 259 |
Anti-CD2 Antibody [RPA-2.10], FITC-25Tests |
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| QAB1-F-25Tests | EnQuireBio | 25Tests | EUR 157 |
Anti-CD2 Antibody [RPA-2.10], FITC-500Tests |
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| QAB1-F-500Tests | EnQuireBio | 500Tests | EUR 953 |
Anti-CD2 Antibody [RPA-2.10], PerCP-100Tests |
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| QAB1-PCP-100Tests | EnQuireBio | 100Tests | EUR 377 |
Anti-CD2 Antibody [RPA-2.10], PerCP-25Tests |
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| QAB1-PCP-25Tests | EnQuireBio | 25Tests | EUR 182 |
Anti-CD2 Antibody [RPA-2.10], PerCP-500Tests |
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| QAB1-PCP-500Tests | EnQuireBio | 500Tests | EUR 1444 |
Anti-CD2 Antibody [RPA-2.10], PE-100Tests |
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| QAB1-PE-100Tests | EnQuireBio | 100Tests | EUR 318 |
Anti-CD2 Antibody [RPA-2.10], PE-25Tests |
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| QAB1-PE-25Tests | EnQuireBio | 25Tests | EUR 182 |
Anti-CD2 Antibody [RPA-2.10], PE-500Tests |
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| QAB1-PE-500Tests | EnQuireBio | 500Tests | EUR 1182 |
Anti-CD2 Antibody [RPA-2.10], V450-100Tests |
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| QAB1-V450-100Tests | EnQuireBio | 100Tests | EUR 411 |
Anti-CD2 Antibody [RPA-2.10], V450-25Tests |
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| QAB1-V450-25Tests | EnQuireBio | 25Tests | EUR 200 |
Anti-CD2 Antibody [RPA-2.10], V450-500Tests |
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| QAB1-V450-500Tests | EnQuireBio | 500Tests | EUR 1596 |
Anti-CD4 Antibody [RPA-T4], Unconjugated-100ug |
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| QAB12-100ug | EnQuireBio | 100ug | EUR 123 |
Anti-CD4 Antibody [RPA-T4], APC-100Tests |
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| QAB12-APC-100Tests | EnQuireBio | 100Tests | EUR 182 |
Anti-CD4 Antibody [RPA-T4], APC-25Tests |
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| QAB12-APC-25Tests | EnQuireBio | 25Tests | EUR 123 |
Anti-CD4 Antibody [RPA-T4], APC-500Tests |
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| QAB12-APC-500Tests | EnQuireBio | 500Tests | EUR 487 |
Anti-CD4 Antibody [RPA-T4], FITC-100Tests |
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| QAB12-F-100Tests | EnQuireBio | 100Tests | EUR 141 |
Anti-CD4 Antibody [RPA-T4], FITC-25Tests |
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| QAB12-F-25Tests | EnQuireBio | 25Tests | EUR 115 |
Anti-CD4 Antibody [RPA-T4], FITC-500Tests |
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| QAB12-F-500Tests | EnQuireBio | 500Tests | EUR 403 |
Anti-CD4 Antibody [RPA-T4], PE-100Tests |
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| QAB12-PE-100Tests | EnQuireBio | 100Tests | EUR 166 |
Anti-CD4 Antibody [RPA-T4], PE-25Tests |
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| QAB12-PE-25Tests | EnQuireBio | 25Tests | EUR 115 |
Anti-CD4 Antibody [RPA-T4], PE-500Tests |
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| QAB12-PE-500Tests | EnQuireBio | 500Tests | EUR 487 |
Anti-CD4 Antibody [RPA-T4], V450-100Tests |
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| QAB12-V450-100Tests | EnQuireBio | 100Tests | EUR 242 |
Anti-CD4 Antibody [RPA-T4], V450-25Tests |
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| QAB12-V450-25Tests | EnQuireBio | 25Tests | EUR 141 |
Anti-CD8a Antibody [RPA-T8], Unconjugated-100ug |
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| QAB19-100ug | EnQuireBio | 100ug | EUR 149 |
Anti-CD8a Antibody [RPA-T8], APC-100Tests |
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| QAB19-APC-100Tests | EnQuireBio | 100Tests | EUR 182 |
Anti-CD8a Antibody [RPA-T8], APC-25Tests |
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| QAB19-APC-25Tests | EnQuireBio | 25Tests | EUR 123 |
Anti-CD8a Antibody [RPA-T8], APC-500Tests |
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| QAB19-APC-500Tests | EnQuireBio | 500Tests | EUR 487 |
Anti-CD8a Antibody [RPA-T8], FITC-100Tests |
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| QAB19-F-100Tests | EnQuireBio | 100Tests | EUR 141 |
Anti-CD8a Antibody [RPA-T8], FITC-25Tests |
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| QAB19-F-25Tests | EnQuireBio | 25Tests | EUR 115 |
Anti-CD8a Antibody [RPA-T8], FITC-500Tests |
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| QAB19-F-500Tests | EnQuireBio | 500Tests | EUR 403 |
Anti-CD8a Antibody [RPA-T8], PE-100Tests |
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| QAB19-PE-100Tests | EnQuireBio | 100Tests | EUR 182 |
Anti-CD8a Antibody [RPA-T8], PE-25Tests |
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| QAB19-PE-25Tests | EnQuireBio | 25Tests | EUR 123 |
Anti-CD8a Antibody [RPA-T8], PE-500Tests |
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| QAB19-PE-500Tests | EnQuireBio | 500Tests | EUR 487 |
Anti-CD8a Antibody [RPA-T8], V450-100Tests |
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| QAB19-V450-100Tests | EnQuireBio | 100Tests | EUR 233 |
Anti-CD8a Antibody [RPA-T8], V450-25Tests |
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| QAB19-V450-25Tests | EnQuireBio | 25Tests | EUR 149 |
Polyclonal Goat Anti-GCIP-interacting protein p29 Antibody |
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| APG00146G | Leading Biology | 0.1mg | EUR 484 |
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Description: A polyclonal antibody raised in Goat that recognizes and binds to Human Goat Anti-GCIP-interacting protein p29 . This antibody is tested and proven to work in the following applications: |
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Huntingtin Interacting Protein 2 (HIP2) Polyclonal Antibody (Human) |
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| 4-PAE601Hu01 | Cloud-Clone |
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Description: A Rabbit polyclonal antibody against Human Huntingtin Interacting Protein 2 (HIP2) |
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Toll Interacting Protein (TOLLIP) Polyclonal Antibody (Human), APC |
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| 4-PAC803Hu01-APC | Cloud-Clone |
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Description: A Rabbit polyclonal antibody against Human Toll Interacting Protein (TOLLIP). This antibody is labeled with APC. |
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Toll Interacting Protein (TOLLIP) Polyclonal Antibody (Human), Biotinylated |
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| 4-PAC803Hu01-Biotin | Cloud-Clone |
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Description: A Rabbit polyclonal antibody against Human Toll Interacting Protein (TOLLIP). This antibody is labeled with Biotin. |
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Toll Interacting Protein (TOLLIP) Polyclonal Antibody (Human), Cy3 |
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| 4-PAC803Hu01-Cy3 | Cloud-Clone |
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Description: A Rabbit polyclonal antibody against Human Toll Interacting Protein (TOLLIP). This antibody is labeled with Cy3. |
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Toll Interacting Protein (TOLLIP) Polyclonal Antibody (Human), FITC |
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| 4-PAC803Hu01-FITC | Cloud-Clone |
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Description: A Rabbit polyclonal antibody against Human Toll Interacting Protein (TOLLIP). This antibody is labeled with FITC. |
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Toll Interacting Protein (TOLLIP) Polyclonal Antibody (Human), HRP |
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| 4-PAC803Hu01-HRP | Cloud-Clone |
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Description: A Rabbit polyclonal antibody against Human Toll Interacting Protein (TOLLIP). This antibody is labeled with HRP. |
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Toll Interacting Protein (TOLLIP) Polyclonal Antibody (Human), PE |
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| 4-PAC803Hu01-PE | Cloud-Clone |
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Description: A Rabbit polyclonal antibody against Human Toll Interacting Protein (TOLLIP). This antibody is labeled with PE. |
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Toll Interacting Protein (TOLLIP) Polyclonal Antibody (Human, Mouse) |
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| 4-PAC803Mu01 | Cloud-Clone |
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Description: A Rabbit polyclonal antibody against Human, Mouse Toll Interacting Protein (TOLLIP) |
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Thyroid receptor interacting protein 15 (TRIP15) polyclonal antibody |
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| ABP-PAB-10139 | Allele Biotech | 100 ug | Ask for price |
Fms interacting protein (FMIP) [domain 1] polyclonal antibody |
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| ABP-PAB-11638 | Allele Biotech | 100 ug | Ask for price |
Fms interacting protein (FMIP) [domain 2] polyclonal antibody |
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| ABP-PAB-11639 | Allele Biotech | 100 ug | Ask for price |
Traf Interacting Protein Antibody |
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| 20-abx116139 | Abbexa |
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HSP70 Interacting Protein Antibody |
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| 20-abx137349 | Abbexa |
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Toll-interacting protein Antibody |
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| 20-abx137443 | Abbexa |
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Notably, the overexpression of cyclin F(S621G) led to the aggregation and altered subcellular distribution of SFPQ in HEK293 cells, while resulting in altered degradation in major neurons. Overall, our knowledge hyperlinks ALS/FTD-causing mutations in CCNF to converging pathological options of ALS/FTD and supplies a hyperlink between faulty protein degradation programs and the pathological accumulation of a protein concerned in RNA processing and metabolism. Once generated in vivo, trans-3MGC acid is proposed to isomerize through a mechanism involving π electron delocalization with formation of a resonance construction that allows bond rotation.