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Rabbit Anti-IARS2 Recombinant Antibody (EG1596) (CBMAB-EN1909-LY)

The product is antibody recognizes IARS2. The antibody EG1596 immunoassay techniques such as: WB: 1:500~1:1000 IHC: 1:50~1:100 IF: 1:100~1:500 ELISA: 1:1000.
See all IARS2 antibodies

Summary

Host Animal
Rabbit
Specificity
Human, Mouse
Clone
EG1596
Antibody Isotype
IgG
Application
WB: 1:500~1:1000 IHC: 1:50~1:100 IF: 1:100~1:500 ELISA: 1:1000

Basic Information

Immunogen
The antibody was produced against synthesized peptide derived from internal of human IARS2.
Specificity
Human, Mouse
Antibody Isotype
IgG
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
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
isoleucyl-tRNA synthetase 2, mitochondrial
Introduction
Aminoacyl-tRNA synthetases catalyze the aminoacylation of tRNA by their cognate amino acid. Because of their central role in linking amino acids with nucleotide triplets contained in tRNAS, aminoacyl-tRNA synthetases are thought to be among the first proteins that appeared in evolution. Two forms of isoleucine-tRNA synthetase exist, a cytoplasmic form and a mitochondrial form. This gene encodes the mitochondrial isoleucine-tRNA synthetase which belongs to the class-I aminoacyl-tRNA synthetase family.
Entrez Gene ID
Human55699
Mouse381314
UniProt ID
HumanQ9NSE4
MouseQ8BIJ6
Alternative Names
ILERS; CAGSSS
Biological Process
Isoleucyl-tRNA aminoacylation Source: GO_Central
Mitochondrial translation Source: GO_Central
tRNA aminoacylation for protein translation Source: Reactome
Cellular Location
Mitochondrion matrix
Involvement in disease
Cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia (CAGSSS):
An autosomal recessive disorder characterized by cataracts, short-stature secondary to growth hormone deficiency, sensorineural hearing deficit, peripheral sensory neuropathy, skeletal dysplasia, scoliosis, and facial dysmorphism.

Gong, Y., Lan, X. P., & Guo, S. (2023). IARS2-related disease manifesting as sideroblastic anemia and hypoparathyroidism: A case report. Frontiers in Pediatrics, 10, 1080664.

Upadia, J., Li, Y., Walano, N., Deputy, S., Gajewski, K., & Andersson, H. C. (2022). Genotype–phenotype correlation in IARS2‐related diseases: A case report and review of literature. Clinical Case Reports, 10(2), e05401.

Barcia, G., Pandithan, D., Ruzzenente, B., Assouline, Z., Pennisi, A., Ormieres, C., ... & Steffann, J. (2021). Biallelic IARS2 mutations presenting as sideroblastic anemia. haematologica, 106(4), 1220.

Ma, D., Li, S., Nie, X., Chen, L., Chen, N., Hou, D., ... & Gao, B. (2020). RNAi-mediated IARS2 knockdown inhibits proliferation and promotes apoptosis in human melanoma A375 cells. Oncology Letters, 20(2), 1093-1100.

Lee, J. S., Kim, M. J., Kim, S. Y., Lim, B. C., Kim, K. J., Choi, M., ... & Chae, J. H. (2019). Clinical and genetic characteristics of Korean patients with IARS2-related disorders. Journal of Genetic Medicine, 16(2), 55-61.

Li, H., Tian, Y., Li, X., Wang, B., Zhai, D., Bai, Y., ... & Chao, X. (2019). Knockdown of IARS2 inhibited proliferation of acute myeloid leukemia cells by regulating p53/p21/PCNA/eIF4E pathway. Oncology Research, 27(6), 673.

Di, X., Jin, X., Ma, H., Wang, R., Cong, S., Tian, C., ... & Wang, K. (2019). The oncogene IARS2 promotes non-small cell lung cancer tumorigenesis by activating the AKT/MTOR pathway. Frontiers in Oncology, 9, 393.

Takezawa, Y., Fujie, H., Kikuchi, A., Niihori, T., Funayama, R., Shirota, M., ... & Kure, S. (2018). Novel IARS2 mutations in Japanese siblings with CAGSSS, Leigh, and West syndrome. Brain and Development, 40(10), 934-938.

Vona, B., Maroofian, R., Bellacchio, E., Najafi, M., Thompson, K., Alahmad, A., ... & Karimiani, E. G. (2018). Expanding the clinical phenotype of IARS2-related mitochondrial disease. BMC Medical Genetics, 19, 1-16.

Fang, Z., Wang, X., Yan, Q., Zhang, S., & Li, Y. (2018). Knockdown of IARS2 suppressed growth of gastric cancer cells by regulating the phosphorylation of cell cycle-related proteins. Molecular and Cellular Biochemistry, 443, 93-100.

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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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