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Mouse Anti-NUP98 Recombinant Antibody (CBT4369) (V2LY-0625-LY1643)

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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBT4369
Antibody Isotype
IgG1
Application
WB, ICC, FC

Basic Information

Immunogen
Purified recombinant fragment of human NUP98 (AA: 1-218) expressed in E. Coli.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1:500-1:2,000
ICC1:200-1:1,000
FC1:200-1:400
ELISA1:10,000

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS
Preservative
Sodium azide
Concentration
Batch dependent
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Nucleoporin 98
Entrez Gene ID
UniProt ID
Function
Plays a role in the nuclear pore complex (NPC) assembly and/or maintenance. NUP98 and NUP96 are involved in the bidirectional transport across the NPC (PubMed:33097660).
May anchor NUP153 and TPR to the NPC. In cooperation with DHX9, plays a role in transcription and alternative splicing activation of a subset of genes (PubMed:28221134).
Involved in the localization of DHX9 in discrete intranuclear foci (GLFG-body) (PubMed:28221134).
(Microbial infection) Interacts with HIV-1 capsid protein P24 and nucleocapsid protein P7 and may thereby promote the integration of the virus in the host nucleus (in vitro) (PubMed:23523133).
Binding affinity to HIV-1 CA-NC complexes bearing the capsid change ASN-74-ASP is reduced (in vitro) (PubMed:23523133).
Biological Process
mRNA transportIEA:UniProtKB-KW
Nuclear pore complex assemblyManual Assertion Based On ExperimentIMP:UniProtKB
Nuclear pore organization1 PublicationNAS:UniProtKB
Nucleocytoplasmic transportManual Assertion Based On ExperimentTAS:UniProtKB
Positive regulation of mRNA splicing, via spliceosomeManual Assertion Based On ExperimentIMP:UniProtKB
Protein import into nucleusIEA:Ensembl
Cellular Location
Nucleus membrane
Nucleus, nuclear pore complex
Nucleus, nucleoplasm
Localized to the nucleoplasmic side of the nuclear pore complex (NPC), at or near the nucleoplasmic basket (PubMed:11839768).
Dissociates from the dissasembled NPC structure early during prophase of mitosis (PubMed:12802065).
Colocalized with NUP153 and TPR to the nuclear basket of NPC (PubMed:11839768).
Colocalized with DHX9 in diffuse and discrete intranuclear foci (GLFG-body) (PubMed:11839768, PubMed:28221134).
(Microbial infection) Remains localized to the nuclear membrane after poliovirus (PV) infection.
Involvement in disease
Chromosomal aberrations involving NUP98 have been found in acute myeloid leukemia. Translocation t(7;11)(p15;p15) with HOXA9 (PubMed:8563753). The chimera includes NUP98 intrinsic disordered regions which contribute to aberrant liquid-liquid phase separation puncta of the chimera in the nucleus. This phase-separation enhances the chimera genomic targeting and induces organization of aberrant three-dimensional chromatin structures leading to tumorous transformation (PubMed:34163069). Translocation t(11;17)(p15;p13) with PHF23 (PubMed:17287853).
A chromosomal aberration involving NUP98 has been found in M0 type acute myeloid leukemia. Translocation t(4;11)(q23;p15) with RAP1GDS1.
A chromosomal aberration involving NUP98 has been found in T-cell acute lymphocytic leukemia. Translocation t(4;11)(q23;p15) with RAP1GDS1.
A chromosomal aberration involving NUP98 has been found in M5 type acute myeloid leukemia. Translocation t(11;12)(p15;p13) with KDM5A.
Chromosomal aberrations involving NUP98 have been found in childhood acute myeloid leukemia. Translocation t(5;11)(q35;p15.5) with NSD1. Translocation t(8;11)(p11.2;p15) with WHSC1L1.
Chromosomal aberrations involving NUP98 have been found in M7 type childhood acute myeloid leukemia. Translocation t(11;12)(p15;p13) with KDM5A.
A chromosomal aberration involving NUP98 is found in a form of therapy-related myelodysplastic syndrome. Translocation t(11;20)(p15;q11) with TOP1.
A chromosomal aberration involving NUP98 is found in a form of T-cell acute lymphoblastic leukemia (T-ALL). Translocation t(3;11)(q12.2;p15.4) with LNP1.
A chromosomal aberration involving NUP98 is associated with pediatric acute myeloid leukemia (AML) with intermediate characteristics between M2-M3 French-American-British (FAB) subtypes. Translocation t(9;11)(p22;p15) with PSIP1/LEDGF. The chimeric transcript is an in-frame fusion of NUP98 exon 8 to PSIP1/LEDGF exon 4.
A chromosomal aberration involving NUP98 has been identified in acute leukemias. Translocation t(6;11)(q24.1;p15.5) with CCDC28A. The chimeric transcript is an in-frame fusion of NUP98 exon 13 to CCDC28A exon 2. Ectopic expression of NUP98-CCDC28A in mouse promotes the proliferative capacity and self-renewal potential of hematopoietic progenitors and rapidly induced fatal myeloproliferative neoplasms and defects in the differentiation of the erythro-megakaryocytic lineage.
PTM
Isoform 1 to isoform 4 are autoproteolytically cleaved to yield Nup98 and Nup96 or Nup98 only, respectively (PubMed:10087256, PubMed:20407419, PubMed:12191480, PubMed:18287282).
Cleaved Nup98 is necessary for the targeting of Nup98 to the nuclear pore and the interaction with Nup96 (PubMed:20407419, PubMed:12191480).
Proteolytically degraded after poliovirus (PV) infection; degradation is partial and NCP- and TPR-binding domains withstand degradation.
More Infomation

Matsukawa, T., Yin, M., Nigam, N., Negi, V., Li, L., Small, D., ... & Aplan, P. D. (2023). NUP98:: Nsd1 and FLT3-ITD collaborate to generate acute myeloid leukemia. Leukemia, 37(7), 1545-1548.

Bertrums, E. J., Smith, J. L., Harmon, L., Ries, R. E., Wang, Y. C. J., Alonzo, T. A., ... & Meshinchi, S. (2023). Comprehensive molecular and clinical characterization of NUP98 fusions in pediatric acute myeloid leukemia. Haematologica, 108(8), 2044.

Ng, S. C., & Görlich, D. (2022). A simple thermodynamic description of phase separation of Nup98 FG domains. Nature Communications, 13(1), 6172.

Chandra, B., Michmerhuizen, N. L., Shirnekhi, H. K., Tripathi, S., Pioso, B. J., Baggett, D. W., ... & Kriwacki, R. W. (2022). Phase separation mediates NUP98 fusion oncoprotein leukemic transformation. Cancer discovery, 12(4), 1152-1169.

Heikamp, E. B., Henrich, J. A., Perner, F., Wong, E. M., Hatton, C., Wen, Y., ... & Armstrong, S. A. (2022). The menin-MLL1 interaction is a molecular dependency in NUP98-rearranged AML. Blood, The Journal of the American Society of Hematology, 139(6), 894-906.

Terlecki-Zaniewicz, S., Humer, T., Eder, T., Schmoellerl, J., Heyes, E., Manhart, G., ... & Grebien, F. (2021). Biomolecular condensation of NUP98 fusion proteins drives leukemogenic gene expression. Nature structural & molecular biology, 28(2), 190-201.

Noort, S., Wander, P., Alonzo, T. A., Smith, J., Ries, R. E., Gerbing, R. B., ... & Meshinchi, S. (2021). The clinical and biological characteristics of NUP98-KDM5A pediatric acute myeloid leukemia. Haematologica, 106(2), 630.

Michmerhuizen, N. L., Klco, J. M., & Mullighan, C. G. (2020). Mechanistic insights and potential therapeutic approaches for NUP98-rearranged hematologic malignancies. Blood, The Journal of the American Society of Hematology, 136(20), 2275-2289.

Schmoellerl, J., Barbosa, I. A. M., Eder, T., Brandstoetter, T., Schmidt, L., Maurer, B., ... & Grebien, F. (2020). CDK6 is an essential direct target of NUP98 fusion proteins in acute myeloid leukemia. Blood, The Journal of the American Society of Hematology, 136(4), 387-400.

Zhang, Y., Guo, Y., Gough, S. M., Zhang, J., Vann, K. R., Li, K., ... & Kutateladze, T. G. (2020). Mechanistic insights into chromatin targeting by leukemic NUP98-PHF23 fusion. Nature Communications, 11(1), 3339.

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For research use only. Not intended for any clinical use.

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