Mouse Anti-NOP10 Recombinant Antibody (6H6) (CBMAB-N2924-WJ)

Name: Mouse Anti-NOP10 Recombinant Antibody (6H6)
SKU: CBMAB-N2924-WJ
Rating: 5 (1 reviews)

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Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

6H6

Antibody Isotype

IgG2a, κ

Application

ELISA, WB

Basic Information

Specificity

Human

Antibody Isotype

IgG2a, κ

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

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

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name

NOP10 ribonucleoprotein homolog (yeast)

Introduction

This gene is a member of the H/ACA snoRNPs (small nucleolar ribonucleoproteins) gene family. snoRNPs are involved in various aspects of rRNA processing and modification and have been classified into two families: C/D and H/ACA. The H/ACA snoRNPs also include the DKC1, NOLA1 and NOLA2 proteins. These four H/ACA snoRNP proteins localize to the dense fibrillar components of nucleoli and to coiled (Cajal) bodies in the nucleus. Both 18S rRNA production and rRNA pseudouridylation are impaired if any one of the four proteins is depleted. The four H/ACA snoRNP proteins are also components of the telomerase complex. This gene encodes a protein related to Saccharomyces cerevisiae Nop10p. [provided by RefSeq, Jul 2008]

Entrez Gene ID

55505

UniProt ID

Q9NPE3

Alternative Names

NOP10 Ribonucleoprotein; Nucleolar Protein Family A, Member 3 (H/ACA Small Nucleolar RNPs); Nucleolar Protein Family A Member 3; Homolog Of Yeast Nop10p; Nucleolar Protein 10; SnoRNP Protein NOP10; NOLA3;

Function

Required for ribosome biogenesis and telomere maintenance. Part of the H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA. This involves the isomerization of uridine such that the ribose is subsequently attached to C5, instead of the normal N1. Each rRNA can contain up to 100 pseudouridine ('psi') residues, which may serve to stabilize the conformation of rRNAs. May also be required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme.

Biological Process

Positive regulation of telomerase RNA localization to Cajal bodyManual Assertion Based On ExperimentHMP:BHF-UCL
Pseudouridine synthesis1 PublicationNAS:UniProtKB
rRNA pseudouridine synthesisManual Assertion Based On ExperimentIBA:GO_Central
snRNA pseudouridine synthesisManual Assertion Based On ExperimentIBA:GO_Central
Telomere maintenance via telomeraseManual Assertion Based On ExperimentIDA:UniProtKB

Cellular Location

Nucleus, nucleolus
Nucleus, Cajal body
Also localized to Cajal bodies (coiled bodies).

Involvement in disease

Dyskeratosis congenita, autosomal recessive, 1 (DKCB1):
A rare multisystem disorder caused by defective telomere maintenance. It is characterized by progressive bone marrow failure, and the clinical triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. Common but variable features include premature graying, aplastic anemia, low platelets, osteoporosis, pulmonary fibrosis, and liver fibrosis among others. Early mortality is often associated with bone marrow failure, infections, fatal pulmonary complications, or malignancy.

More Infomation

References

Monteagudo, M., Martínez, P., Leandro-García, L. J., Martínez-Montes, Á. M., Calsina, B., Pulgarín-Alfaro, M., ... & Robledo, M. (2021). Analysis of telomere maintenance related genes reveals NOP10 as a new metastatic-risk marker in pheochromocytoma/paraganglioma. Cancers, 13(19), 4758.

Elsharawy, K. A., Althobiti, M., Mohammed, O. J., Aljohani, A. I., Toss, M. S., Green, A. R., & Rakha, E. A. (2021). Nucleolar protein 10 (NOP10) predicts poor prognosis in invasive breast cancer. Breast cancer research and treatment, 185, 615-627.

Cui, C., Liu, Y., Gerloff, D., Rohde, C., Pauli, C., Koehn, M., ... & Mueller-Tidow, C. (2021). NOP10 predicts lung cancer prognosis and its associated small nucleolar RNAs drive proliferation and migration. Oncogene, 40(5), 909-921.

Ibáñez-Cabellos, J. S., Seco-Cervera, M., Picher-Latorre, C., Pérez-Machado, G., García-Giménez, J. L., & Pallardó, F. V. (2020). Acute depletion of telomerase components DKC1 and NOP10 induces oxidative stress and disrupts ribosomal biogenesis via NPM1 and activation of the P53 pathway. Biochimica et Biophysica Acta (BBA)-Molecular Cell Research, 1867(12), 118845.

Balogh, E., Chandler, J. C., Varga, M., Tahoun, M., Menyhárd, D. K., Schay, G., ... & Tory, K. (2020). Pseudouridylation defect due to DKC1 and NOP10 mutations causes nephrotic syndrome with cataracts, hearing impairment, and enterocolitis. Proceedings of the National Academy of Sciences, 117(26), 15137-15147.

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Protocols

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Associated Products

Isotype control

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

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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Documents

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SDS
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Second antibody and isotype control

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