Recombinant Human Probable leucine--tRNA ligase, mitochondrial (LARS2), partial

Code CSB-YP613580HU
MSDS
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Source Yeast
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Code CSB-EP613580HU
MSDS
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Source E.coli
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Code CSB-EP613580HU-B
MSDS
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Source E.coli
Conjugate Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
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Code CSB-BP613580HU
MSDS
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Source Baculovirus
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Code CSB-MP613580HU
MSDS
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Source Mammalian cell
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Product Details

Purity
>85% (SDS-PAGE)
Target Names
LARS2
Uniprot No.
Alternative Names
HLASA; KIAA0028; LARS 2; Lars2; Leucine translase; Leucine tRNA ligase 2; Leucine tRNA ligase 2 mitocondrial; Leucine tRNA ligase; Leucine--tRNA ligase; Leucyl tRNA synthetase 2; Leucyl tRNA synthetase 2 mitochondrial; LeuRS; MGC26121; mtLeuRS; PRLTS4; Probable leucyl-tRNA synthetase, mitochondrial; SYLM_HUMAN
Species
Homo sapiens (Human)
Expression Region
-
Protein Length
Partial
Tag Info
Tag type will be determined during the manufacturing process.
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Form
Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer before Lyophilization
Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Reconstitution
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting and FAQs
Storage Condition
Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time
Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet
Please contact us to get it.

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Target Background

Gene References into Functions
  1. IARS2 knockdown inhibits proliferation, suppresses colony formation, and causes cell cycle arrest in AGS cells. PMID: 29071539
  2. ere we present eight families affected by Perrault syndrome. In five families we identified novel or previously reported variants in HSD17B4, LARS2, CLPP and C10orf2 PMID: 26970254
  3. This represents the first independent replication of the involvement of LARS2 mutations in Perrault syndrome, contributing valuable information for the further understanding of this disease PMID: 26657938
  4. analysis of the CP1 domain in human mitochondrial leucyl-tRNA synthetase PMID: 26272616
  5. Leucyl tRNA synthetase is able to partially rescue defects caused by mutations in non-cognate itochondrial-tRNAs. PMID: 24413189
  6. Mutations in LARS2, encoding mitochondrial leucyl-tRNA synthetase, lead to premature ovarian failure and hearing loss in Perrault syndrome. PMID: 23541342
  7. No evidence to support previous data indicating a role in type 2 diabetes susceptibility in humans with LARS2 single nucleotide polymorphisms PMID: 19847392
  8. The alteration of aminoacylation tRNA(Leu(UUR)) caused by the A3243G mutation led to mitochondrial translational defects and thereby reduced the aminoacylated efficiencies of tRNA(Leu(UUR)) as well as tRNA(Ala) and tRNA(Met). PMID: 20194621
  9. Upregulation of LARS2 is a hallmark of 324A>G mutation. The accumulation of 3243A>G mutation in the brain may have a pathophysiologic role in bipolar disorder and schizophrenia. PMID: 15737668
  10. In this study, we provide evidence that the LARS2 gene may represent a novel type 2 diabetes susceptibility gene. PMID: 15919814
  11. There was investigated whether overexpression of human mitochondrial LeuRS suppressed translation and respiratory chain defects associated with the pathogenic A3243G mutation in human cells. PMID: 18796578
  12. data indicate that inactivation of LARS2 by both genetic and epigenetic mechanisms may be a common and important event in the carcinogenesis of nasopharyngeal carcinoma PMID: 19129950

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Involvement in disease
Perrault syndrome 4 (PRLTS4); Hydrops, lactic acidosis, and sideroblastic anemia (HLASA)
Subcellular Location
Mitochondrion matrix.
Protein Families
Class-I aminoacyl-tRNA synthetase family
Tissue Specificity
Ubiquitously expressed, but highest expression in tissues with high metabolic rates, such as skeletal muscle, heart, and kidney.
Database Links

HGNC: 17095

OMIM: 604544

KEGG: hsa:23395

STRING: 9606.ENSP00000265537

UniGene: Hs.526975

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