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Mouse Anti-LARS2 Recombinant Antibody (31C2a) (CBMAB-L0699-YJ)

Provided herein is a Mouse monoclonal antibody, which binds to Leucyl-Trna Synthetase 2 Mitochondrial (LARS2). The antibody can be used for immunoassay techniques, such as Dot, ICC.
See all LARS2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
31C2a
Antibody Isotype
IgG1
Application
Dot, ICC

Basic Information

Specificity
Human
Antibody Isotype
IgG1
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!]

Buffer
PBS, pH 7.2, 1% BSA
Preservative
0.05% Sodium Azide
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
leucyl-tRNA synthetase 2, mitochondrial
Introduction
LARS2 is a class 1 aminoacyl-tRNA synthetase, mitochondrial leucyl-tRNA synthetase. Each of the twenty aminoacyl-tRNA synthetases catalyzes the aminoacylation of a specific tRNA or tRNA isoaccepting family with the cognate amino acid. Diseases associated with LARS2 include Hydrops, Lactic Acidosis, And Sideroblastic Anemia and Perrault Syndrome 4. Among its related pathways are tRNA Aminoacylation and Amino Acid metabolism.
Entrez Gene ID
23395
UniProt ID
Q15031
Function
Catalyzes the attachment of leucine to its cognate tRNA.
Biological Process
Leucyl-tRNA aminoacylationManual Assertion Based On ExperimentIDA:HGNC
Mitochondrial translationManual Assertion Based On ExperimentIBA:GO_Central
tRNA aminoacylation for protein translationTAS:Reactome
Cellular Location
Mitochondrion matrix
Involvement in disease
Perrault syndrome 4 (PRLTS4):
An autosomal recessive, sex-influenced disorder characterized by sensorineural deafness in both males and females, and ovarian dysgenesis in females. Affected females have primary amenorrhea, streak gonads, and infertility, whereas affected males show normal pubertal development and are fertile.
Hydrops, lactic acidosis, and sideroblastic anemia (HLASA):
A lethal, multisystem metabolic disorder characterized by severe lactic acidosis, hydrops, and sideroblastic anemia. Additional features include impaired cardiac function, disordered coagulation, pulmonary hypertension, and progressive renal disease.

Neyroud, A. S., Rudinger-Thirion, J., Frugier, M., Riley, L. G., Bidet, M., Akloul, L., ... & Jaillard, S. (2023). LARS2 variants can present as premature ovarian insufficiency in the absence of overt hearing loss. European Journal of Human Genetics, 31(4), 453-460.

Vona, B. (2023). An encounter with the mild side of LARS2-associated Perrault syndrome and its implications on the diagnostic odyssey. European Journal of Human Genetics, 1-2.

Feng, S., Wan, S., Liu, S., Wang, W., Tang, M., Bai, L., & Zhu, Y. (2022). LARS2 regulates apoptosis via ROS-mediated mitochondrial dysfunction and endoplasmic reticulum stress in ovarian granulosa cells. Oxidative Medicine and Cellular Longevity, 2022.

Capriglia, F., Rizzo, F., Petrosillo, G., Morea, V., d’Amati, G., Cantatore, P., ... & Bruni, F. (2021). Exploring the ability of LARS2 carboxy-terminal domain in rescuing the MELAS phenotype. Life, 11(7), 674.

Riley, L. G., Rudinger‐Thirion, J., Frugier, M., Wilson, M., Luig, M., Alahakoon, T. I., ... & Christodoulou, J. (2020). The expanding LARS2 phenotypic spectrum: HLASA, Perrault syndrome with leukodystrophy, and mitochondrial myopathy. Human mutation, 41(8), 1425-1434.

Carminho-Rodrigues, M. T., Klee, P., Laurent, S., Guipponi, M., Abramowicz, M., Cao-van, H., ... & Paoloni-Giacobino, A. (2020). LARS2-Perrault syndrome: a new case report and literature review. BMC medical genetics, 21, 1-9.

Tucker, E. J., Rius, R., Jaillard, S., Bell, K., Lamont, P. J., Travessa, A., ... & Sinclair, A. H. (2020). Genomic sequencing highlights the diverse molecular causes of Perrault syndrome: a peroxisomal disorder (PEX6), metabolic disorders (CLPP, GGPS1), and mtDNA maintenance/translation disorders (LARS2, TFAM). Human genetics, 139, 1325-1343.

van Der Knaap, M. S., Bugiani, M., Mendes, M. I., Riley, L. G., Smith, D. E., Rudinger-Thirion, J., ... & Mochel, F. (2019). Biallelic variants in LARS2 and KARS cause deafness and (ovario) leukodystrophy. Neurology, 92(11), e1225-e1237.

Kosaki, R., Horikawa, R., Fujii, E., & Kosaki, K. (2018). Biallelic mutations in LARS2 can cause Perrault syndrome type 2 with neurologic symptoms. American Journal of Medical Genetics Part A, 176(2), 404-408.

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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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