Phenylalanine-tRNA Synthetase 2 (Mitochondrial) Proteine (FARS2)

Aminoacyl-tRNA synthetases are a class of enzymes that charge tRNAs with their cognate amino acids. Zusätzlich bieten wir Ihnen Phenylalanine-tRNA Synthetase 2 (Mitochondrial) Antikörper (29) und und viele weitere Produktgruppen zu diesem Protein an.

alle Proteine anzeigen Gen GeneID UniProt
FARS2 69955 Q99M01
FARS2 10667 O95363
FARS2 306879 Q6AYQ3
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Wheat germ Human GST tag 2 μg 11 bis 12 Tage
$338.33
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HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg 11 Days
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Escherichia coli (E. coli) Human His tag 50 μg 15 bis 19 Tage
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Escherichia coli (E. coli) Human T7 tag,His tag 100 μg 15 bis 18 Tage
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Escherichia coli (E. coli) Maus T7 tag,His tag 100 μg 15 bis 18 Tage
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Escherichia coli (E. coli) Ratte T7 tag,His tag 100 μg 15 bis 18 Tage
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FARS2 Proteine nach Spezies und Herkunft

Origin Exprimiert in Konjugat
Mouse (Murine)

Human , ,
, , ,
Rat (Rattus)

Weitere Proteine zu Phenylalanine-tRNA Synthetase 2 (Mitochondrial) (FARS2) Interaktionspartnern

Human Phenylalanine-tRNA Synthetase 2 (Mitochondrial) (FARS2) Interaktionspartner

  1. The total number of unique pathogenic FARS2 variants known to date is 21 including three different partial gene deletions reported in four individuals. Except for the large deletions, all variants but two (one in-frame deletion of one amino acid and one splice-site variant) are missense. All large deletions and the single splice-site variant are in trans with a missense variant.

  2. Based on the phenotypic data of previously reported subjects and the two subjects reported here, we conclude that FARS2 deficiency can be associated with two phenotypes: (i) an epileptic phenotype, and (ii) a spastic paraplegia phenotype.

  3. in patients with drug-resistant infantile spasm syndrome, associated with focal seizures, mild metabolic changes, and cerebral atrophy with volume loss of white matter on MRI, mutations in FARS2 should be considered.

  4. Kinetic and structural changes in HsmtPheRS, induced by pathogenic mutations in human FARS2 have been described.

  5. A newly identified missense mutation in FARS2 causes autosomal-recessive spastic paraplegia.

  6. this study expands the phenotypic spectrum of FARS2 related disease and emphasizes intragenic deletion in the list of causative mutations.

  7. the three FARS2 mutations directly impair aminoacylation function and stability of mtPheRS, leading to a decrease in overall tRNA charging capacity.

  8. Two phenylalanyl-tRNA synthetase variants Ser57Cys and Asp280Ser both display wild-type aminoacylation activity and stability with respect to their free energies of unfolding, but are less stable at low hydrogen-ion concentration (pH).

  9. The recombinant human enzyme has been purified to homogeneity and crystallized in complex with phenylalanine and ATP.

  10. Formation of the PheRS-tRNAPhe complex in human mitochondria must be accompanied by considerable rearrangement of the anticodon binding domain upon tRNA binding.

  11. Mitochondrial and cytoplasmic phenylalanyl-tRNA synthetases (HsmtPheRS and HsctPheRS, respectively) catalyze direct attachment of m-Tyr to tRNA(Phe).

  12. these results indicate that conformational flexibility of the two functional modules in mtPheRS is essential for its phenylalanylation activity.

Phenylalanine-tRNA Synthetase 2 (Mitochondrial) (FARS2) Protein Überblick

Protein Überblick

Aminoacyl-tRNA synthetases are a class of enzymes that charge tRNAs with their cognate amino acids. This gene encodes a phenylalanine-tRNA synthetase (PheRS) localized to the mitochondrion which consists of a single polypeptide chain, unlike the (alpha-beta)2 structure of the prokaryotic and eukaryotic cytoplasmic forms of PheRS. Structure analysis and catalytic properties indicate mitochondrial PheRSs may constitute a class of PheRS distinct from the enzymes found in prokaryotes and in the eukaryotic cytoplasm.

Genbezeichner und Symbole assoziert mit FARS2

  • phenylalanine-tRNA synthetase 2 (mitochondrial) (Fars2)
  • phenylalanyl-tRNA synthetase 2, mitochondrial (FARS2)
  • phenylalanyl-tRNA synthetase 2, mitochondrial (Fars2)
  • 2810431B21Rik Protein
  • 6720478K01Rik Protein
  • COXPD14 Protein
  • dJ520B18.2 Protein
  • FARS1 Protein
  • HSPC320 Protein
  • PheRS Protein

Bezeichner auf Proteinebene für FARS2

pheRS , phenylalanine--tRNA ligase, mitochondrial , phenylalanine-tRNA synthetase 1 (mitochondrial) , phenylalanyl-tRNA synthetase, mitochondrial , dJ236A3.1 (phenylalanine-tRNA synthetase) , dJ520B18.2 (FARS1 (phenylalanine-tRNA synthetase)) , phenylalanine tRNA ligase 2, mitochondrial , phenylalanine translase , phenylalanine-tRNA synthetase 2 (mitochondrial)

GENE ID SPEZIES
69955 Mus musculus
10667 Homo sapiens
488204 Canis lupus familiaris
505390 Bos taurus
306879 Rattus norvegicus
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