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Rabbit Anti-QRSL1 Recombinant Antibody (RB37609) (CBMAB-1880-YC)


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Summary

Host Animal
Rabbit
Specificity
Human
Clone
RB37609
Antibody Isotype
IgG
Application
ELISA, WB

Basic Information

Immunogen
A KLH conjugated synthetic peptide between 129-156 amino acids from the N-terminal region of human QRSL1
Specificity
Human
Antibody Isotype
IgG
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!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
Localized in a.a.129-156

Target

Full Name
Glutaminyl-TRNA Synthase (Glutamine-Hydrolyzing)-Like 1
Introduction
QRSL belongs to the amidase family. QRSL1 allows the formation of correctly charged Gln-tRNA(Gln) through the transamidation of misacylated Glu-tRNA(Gln) in the mitochondria.
Entrez Gene ID
55278
UniProt ID
Q9H0R6
Alternative Names
GatA
Function
Allows the formation of correctly charged Gln-tRNA(Gln) through the transamidation of misacylated Glu-tRNA(Gln) in the mitochondria. The reaction takes place in the presence of glutamine and ATP through an activated gamma-phospho-Glu-tRNA(Gln).
Biological Process
Biological Process glutaminyl-tRNAGln biosynthesis via transamidationManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process mitochondrial translationManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process regulation of protein stabilityIEA:Ensembl
Cellular Location
Mitochondrion
Involvement in disease
Combined oxidative phosphorylation deficiency 40 (COXPD40):
An autosomal recessive mitochondrial disorder characterized by prenatal or infantile onset, fetal hydrops, severe hypertrophic cardiomyopathy, poor growth, sensorineural hearing loss, hepatic dysfunction, lactic acidosis, and decreased activities of mitochondrial respiratory complexes I, III, IV, and V. The disorder is lethal, with death occurring in infancy.
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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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