QRSL1 Antibody

Code CSB-PA019142GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
QRSL1
Alternative Names
QRSL1 antibody; Glutamyl-tRNA(Gln) amidotransferase subunit A antibody; mitochondrial antibody; Glu-AdT subunit A antibody; EC 6.3.5.7 antibody; Glutaminyl-tRNA synthase-like protein 1 antibody
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Human QRSL1
Immunogen Species
Homo sapiens (Human)
Isotype
IgG
Purification Method
Antigen Affinity purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
ELISA,WB
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

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).
Gene References into Functions
  1. Homozygous intronic variant (c.850-3A > G) in the QRSL1 was identified in a child with cardiomyopathy with early-onset brain disease. PMID: 29440775
  2. Studies showed in vitro Gln-tRNA(Gln) formation catalyzed by the recombinant mtGluRS and hGatCAB. PMID: 19805282
Involvement in disease
Defects in QRSL1 may play a role in mitochondrial disorders characterized by combined respiratory chain complex deficiencies.
Subcellular Location
Mitochondrion.
Protein Families
Amidase family, GatA subfamily
Database Links

HGNC: 21020

KEGG: hsa:55278

STRING: 9606.ENSP00000358042

UniGene: Hs.406917

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