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BCP01756's Information

CAS NO. 775304-57-9 structure
Product Name Translarna
Synonyms PTC-124;PTC 124;PTC124:Translarna
Formula C15H9FN2O3
Molecular Weight 284.24
Spec / Purity 98%
Supply Capacity can supply large quantity in kgs scale
Product Description PTC124 is CFTR-G542X nonsense allele inhibitor.
Details Compared with Gentamicin which is only active at much higher concentrations, PTC124 is a more potent nonsense-suppressing agent and exhibits 4- to 15-fold stimulation of read-through relative to controls. PTC124 (0.01-3 μM) promotes dose-dependent read-through of all three nonsense codons in HEK293 cells harboring LUC-190 nonsense alleles with the highest read-through at UGA, followed by UAG and then UAA, but it does not suppress multiple proximal nonsense codons. Like Gentamicin, PTC124 is most active when a pyrimidine (in particular cytosine, C) follows the nonsense codon. Consistent with the stable cell line reporter assay, PTC124 (17 μM) promotes significant production of dystrophin in primary muscle cells from Duchenne muscular dystrophy (DMD) patients or MDXMDX mice expressing dystrophin nonsense alleles. PTC124 selectively promotes ribosomal read-through of premature termination but not normal termination codons, even at concentrations substantially greater than the values achieving maximal activity.

Price Info Please inquire us for bulk quantity, thanks.
size Price Stock
25 MG
US$85.00
Maintained in stock
1 G
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Maintained in stock
10 G
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Maintained in stock
100 G
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Quality Control
Batch No. File Type File Name
2014042902
LCMS
2014031007
LCMS
2014031903
LCMS
2014031007
NMR
2014031903
NMR
2014042902
NMR
0000
DATASHEET
2014042902
COA
2013122301
COA
2014031903
COA
2015012801
LCMS
2015012801
NMR
2013122301
NMR
2014120801
NMR
2014120801
LCMS
2015031907
LCMS
2015120803
LCMS
2016060613
LCMS
2015120803
NMR
2016030707
LCMS
2015121606
NMR
2016030707
NMR
2015121506
LCMS
2016060613
NMR
2016080202
NMR
2017041708
NMR
2017030103
LCMS
2017030103
NMR
2017031404
NMR

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