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Mouse Anti-ELAC2 (AA 2-101) Recombinant Antibody (CBFYE-0195) (CBMAB-E0439-FY)

This product is mouse antibody that recognizes ELAC2. The antibody CBFYE-0195 can be used for immunoassay techniques such as: ELISA, IF.
See all ELAC2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYE-0195
Antibody Isotype
IgG2a, κ
Application
ELISA, IF

Basic Information

Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
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!]

Format
Liquid
Buffer
PBS, pH 7.2
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
AA 2-101

Target

Full Name
ElaC Ribonuclease Z 2
Introduction
The protein encoded by this gene has a C-terminal domain with tRNA 3′ processing endoribonuclease activity, which catalyzes the removal of the 3' trailer from precursor tRNAs. The protein also interacts with activated Smad family member 2 (Smad2) and its nuclear partner forkhead box H1 (also known as FAST-1), and reduced expression can suppress transforming growth factor-beta induced growth arrest. Mutations in this gene result in an increased risk of prostate cancer. Multiple transcript variants encoding different isoforms have been found for this gene.
Entrez Gene ID
UniProt ID
Alternative Names
ElaC Ribonuclease Z 2; Heredity Prostate Cancer Protein 2; ElaC Homolog Protein 2; TRNA 3 Endonuclease 2; TRNase Z (Long Form); Ribonuclease Z 2; EC 3.1.26.11; TRNase Z 2; RNase Z 2; HPc2
Research Area
Zinc phosphodiesterase, which displays mitochondrial tRNA 3'-processing endonuclease activity. Involved in tRNA maturation, by removing a 3'-trailer from precursor tRNA (PubMed:21593607).

Associates with mitochondrial DNA complexes at the nucleoids to initiate RNA processing and ribosome assembly (PubMed:24703694).
Biological Process
Mitochondrial tRNA 3'-trailer cleavage, endonucleolytic Source: UniProtKB
Mitochondrial tRNA processing Source: Reactome
tRNA 3'-end processing Source: Reactome
tRNA catabolic process Source: Reactome
Cellular Location
Mitochondrion; Mitochondrion nucleoid; Nucleus. Mainly mitochondrial.
Involvement in disease
Prostate cancer, hereditary, 2 (HPC2):
A condition associated with familial predisposition to cancer of the prostate. Most prostate cancers are adenocarcinomas that develop in the acini of the prostatic ducts. Other rare histopathologic types of prostate cancer that occur in approximately 5% of patients include small cell carcinoma, mucinous carcinoma, prostatic ductal carcinoma, transitional cell carcinoma, squamous cell carcinoma, basal cell carcinoma, adenoid cystic carcinoma (basaloid), signet-ring cell carcinoma and neuroendocrine carcinoma.
Combined oxidative phosphorylation deficiency 17 (COXPD17):
An autosomal recessive disorder of mitochondrial dysfunction characterized by onset of severe hypertrophic cardiomyopathy in the first year of life. Other features include hypotonia, poor growth, lactic acidosis, and failure to thrive. The disorder may be fatal in early childhood.

Mendes, L. C., de Oliveira Magalhães, R., Dos Santos, R. K. P., & Araújo, R. S. (2022). Pseudohypoaldosteronism associated with hypertrophic cardiomyopathy, hypertension and thrombocytosis due to mutation in the ELAC2 gene: a case report. Journal of Pediatric Endocrinology and Metabolism.

Migunova, E., Theophilopoulos, J., Mercadante, M., Men, J., Zhou, C., & Dubrovsky, E. B. (2022). Correction: ELAC2/RNaseZ-linked cardiac hypertrophy in Drosophila melanogaster. Disease Models & Mechanisms, 15(3).

Migunova, E., Theophilopoulos, J., Mercadante, M., Men, J., Zhou, C., & Dubrovsky, E. B. (2021). ELAC2/RNaseZ-linked cardiac hypertrophy in Drosophila melanogaster. Disease models & mechanisms, 14(8), dmm048931.

Choi, E. J., Wu, W., Zhang, K., Lee, I., Kim, I. H., Lee, Y. S., & Bao, X. (2021). ELAC2, an enzyme for tRNA maturation, plays a role in the cleavage of a mature tRNA to produce a tRNA-derived RNA fragment during respiratory syncytial virus infection. Frontiers in molecular biosciences, 7, 609732.

Djomkam, A. L. Z., Beyeme Sala, T., Baari Memba, C., & Njimoh, D. L. (2019). Prevalence of the Ser217Leu variant of the ELAC2 gene and its association with prostate cancer in population of the Littoral region of Cameroon. Prostate Cancer, 2019.

Saoura, M., Powell, C. A., Kopajtich, R., Alahmad, A., AL‐Balool, H. H., Albash, B., ... & Minczuk, M. (2019). Mutations in ELAC2 associated with hypertrophic cardiomyopathy impair mitochondrial tRNA 3′‐end processing. Human mutation, 40(10), 1731-1748.

Schroeder, C., Navid-Hill, E., Meiners, J., Hube-Magg, C., Kluth, M., Makrypidi-Fraune, G., ... & Höflmayer, D. (2019). Nuclear ELAC2 overexpression is associated with increased hazard for relapse after radical prostatectomy. Oncotarget, 10(48), 4973.

Paucar, M., Pajak, A., Freyer, C., Bergendal, Å., Döry, M., Laffita-Mesa, J. M., ... & Svenningsson, P. (2018). Chorea, psychosis, acanthocytosis, and prolonged survival associated with ELAC2 mutations. Neurology, 91(15), 710-712.

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

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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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