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

Name: Rabbit Anti-IARS2 Recombinant Antibody (EG1596)
SKU: CBMAB-EN1909-LY
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Rabbit

Clone

EG1596

Application

WB: 1:500~1:1000 IHC: 1:50~1:100 IF: 1:100~1:500 ELISA: 1:1000

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.

More Infomation

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

Human	55699
Mouse	381314

UniProt ID

Human	Q9NSE4
Mouse	Q8BIJ6

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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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)
Proteogenomic
Other Protocols

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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