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Mouse Anti-MARS1 (AA 801-900) Recombinant Antibody (CBFYM-0507) (CBMAB-M0635-FY)

This product is mouse antibody that recognizes MARS. The antibody CBFYM-0507 can be used for immunoassay techniques such as: ELISA, ICC, WB.
See all MARS1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYM-0507
Antibody Isotype
IgG1
Application
ELISA, ICC, WB

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!]

Format
Liquid
Buffer
PBS, pH 7.2
Preservative
0.09% 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.
Epitope
AA 801-900

Target

Full Name
MARS1
Introduction
This gene encodes a member of the class I family of aminoacyl-tRNA synthetases. These enzymes play a critical role in protein biosynthesis by charging tRNAs with their cognate amino acids. The encoded protein is a component of the multi-tRNA synthetase complex and catalyzes the ligation of methionine to tRNA molecules.
Entrez Gene ID
4141
UniProt ID
P56192
Alternative Names
Methionyl-TRNA Synthetase; EC 6.1.1.10; METRS; Methionine TRNA Ligase 1, Cytoplasmic; Methionine--TRNA Ligase, Cytoplasmic; Cytosolic Methionyl-TRNA Synthetase; CMT2U
Function
Catalyzes the specific attachment of an amino acid to its cognate tRNA in a 2 step reaction: the amino acid (AA) is first activated by ATP to form AA-AMP and then transferred to the acceptor end of the tRNA (PubMed:11714285).

Plays a role in the synthesis of ribosomal RNA in the nucleolus (PubMed:10791971).
Biological Process
Cellular response to epidermal growth factor stimulus Source: CAFA
Cellular response to insulin stimulus Source: CAFA
Cellular response to platelet-derived growth factor stimulus Source: CAFA
Cellular response to starvation Source: CAFA
Methionyl-tRNA aminoacylation Source: UniProtKB
Positive regulation of transcription of nucleolar large rRNA by RNA polymerase I Source: CAFA
rRNA transcription Source: CAFA
tRNA aminoacylation for protein translation Source: GO_Central
Cellular Location
Cytoplasm and Cytosol
Nucleus
Nucleolus
Note: Localizes to the nucleolus in proliferative cells but disappears in quiescent cells.
Involvement in disease
Interstitial lung and liver disease (ILLD):
An autosomal recessive, life-threatening disorder characterized by respiratory insufficiency and progressive liver disease with onset in infancy or early childhood. Clinical features include failure to thrive, hypotonia, intermittent lactic acidosis, aminoaciduria, hypothyroidism, interstitial lung disease, pulmonary alveolar proteinosis, anemia, and liver canalicular cholestasis, steatosis, and iron deposition.
Charcot-Marie-Tooth disease 2U (CMT2U):
An axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Neuropathies of the CMT2 group are characterized by signs of axonal degeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. CMT2U is a slowly progressive, autosomal dominant form characterized by late-adult onset.

Wang, A., Song, Z., Zhang, X., Xiao, L., Feng, Y., Qi, C., ... & Wang, F. (2023). MARS1 mutations linked to familial trigeminal neuralgia via the integrated stress response. The Journal of Headache and Pain, 24(1), 4.

Castaldo, A., Delestrain, C., Diesler, R., du Vignaux, C. M., Onnee, M., Touraine, R., ... & De Becdelièvre, A. (2023). Idiopathic pulmonary fibrosis with benign SFTPC variant and pathogenic MARS1 mutations: can't see the forest for the trees!. ERJ Open Research, 9(6).

Botta, E., Theil, A. F., Raams, A., Caligiuri, G., Giachetti, S., Bione, S., ... & Vermeulen, W. (2021). Protein instability associated with AARS1 and MARS1 mutations causes trichothiodystrophy. Human Molecular Genetics, 30(18), 1711-1720.

La Fay, C., Hoebeke, C., Juzaud, M., Spraul, A., Heux, P., Dubus, J. C., ... & Fabre, A. (2021). Deep phenotyping of MARS1 (interstitial lung and liver disease) and LARS1 (infantile liver failure syndrome 1) recessive multisystemic disease using Human Phenotype Ontology annotation: Overlap and differences. Case report and review of literature. European Journal of Medical Genetics, 64(11), 104334.

Lenz, D., Stahl, M., Seidl, E., Schöndorf, D., Brennenstuhl, H., Gesenhues, F., ... & Griese, M. (2020). Rescue of respiratory failure in pulmonary alveolar proteinosis due to pathogenic MARS1 variants. Pediatric Pulmonology, 55(11), 3057-3066.

Perlaza, K., Toutkoushian, H., Boone, M., Lam, M., Iwai, M., Jonikas, M. C., ... & Ramundo, S. (2019). The Mars1 kinase confers photoprotection through signaling in the chloroplast unfolded protein response. Elife, 8, e49577.

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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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