QRSL1 Antibody

Code CSB-PA887948ESR2HU
Size US$166
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  • Immunohistochemistry of paraffin-embedded human small intestine tissue using CSB-PA887948ESR2HU at dilution of 1:100

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) QRSL1 Polyclonal antibody
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
Immunogen
Recombinant Human Glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial protein (289-528AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Clonality
Polyclonal
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, pH7.3.
Form
Liquid
Tested Applications
ELISA, IHC
Recommended Dilution
Application Recommended Dilution
IHC 1:20-1:200
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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