BCH001

本公司产品仅用于科研实验，不得用于人体或动物的临床与诊断

BCH001是PAPD5的特异性小分子抑制剂。BCH001是一种特异性PAPD5抑制剂，可恢复DC患者诱导的多能干细胞中的端粒酶活性和端粒长度。

BCH001 is a specific small-molecule inhibitor of PAPD5.BCH001, a specific PAPD5 inhibitor that restored telomerase activity and telomere length in DC patient induced pluripotent stem cells.?When human blood stem cells engineered to carry DC-causing PARN mutations were xenotransplanted into immunodeficient mice, oral treatment with a repurposed PAPD5 inhibitor, the dihydroquinolizinone RG7834, rescued TERC 3' end maturation and telomere length.?

BCH001 (100 nM-1 μM; for 7 days) reduces telomerase RNA component (TERC) RNA oligo-adenylation and increases steady state TERC RNA levels in PARN-mutant iPSC clones. BCH001 causes few transcriptome-wide changes and rescues other ncRNAs disrupted by PARN mutations. BCH001 (1 μM, 24-72 h) has no adverse impact on cell growth, cell cycle, or apoptosis in induced pluripotent stem cells (iPSCs) from DC.BCH001 inhibits ATP- and dose-dependently recombinant PAPD5 (rPAPD5) in vitro. PAPD5 is a non-canonical polymerase that oligoadenylates and destabilizes telomerase RNA component (TERC).

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Cell Cycle/DNA Damage

DNA/RNA Synthesis

PAPD5

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384859-58-9

420.34

C20H15F3N2O5

>98% (HPLC)

DMSO:84 mg/mL (199.84 mM)

CCOC(=O)c1cnc2ccc(OC(F)(F)F)cc2c1Nc1ccccc1C(O)=O

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(-20℃)

With Ice Pack

≥ 2 years