Mouse Anti-MARS1 Recombinant Antibody (5G5) (CBMAB-A5309-LY)

Name: Mouse Anti-MARS1 Recombinant Antibody (5G5)
SKU: CBMAB-A5309-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

Mouse

Clone

5G5

Application

WB, ELISA

Immunogen

MARS (NP_004981, 801 a.a. ~ 899 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Specificity

Human

Antibody Isotype

IgG1, 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

Purity

> 95% Purity determined by SDS-PAGE.

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

MARS1

Introduction

Aminoacyl-tRNA synthetases are a class of enzymes that charge tRNAs with their cognate amino acids. The protein encoded by this gene belongs to the class I family of tRNA synthetases. [provided by RefSeq]

Entrez Gene ID

4141

UniProt ID

P56192

Alternative Names

FLJ35667; METRS; MTRNS

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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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

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Isotype control

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

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