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Cat. No. | Product Name | Target | Signaling Pathways |
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T12935 |
SMN-C3
MV8T2MCK57 |
DNA/RNA Synthesis | Cell Cycle/Checkpoint; DNA Damage/DNA Repair |
SMN-C3 (MV8T2MCK57) 是口服有效的 SMN2 剪接调节剂。SMN-C3可用于脊椎肌肉萎缩 (SMA) 的研究。 | |||
T73475 |
SMN-C2
|
DNA/RNA Synthesis | Cell Cycle/Checkpoint; DNA Damage/DNA Repair |
SMN-C2 是一种选择性的 SMN2 基因剪接调节剂,是risdiplam类似物,是调节前 mRNA 剪接的选择性 RNA 结合配体,通过结合 SMN2 pre-mRNA 来发挥作用。SMN-C2 具有研究脊髓性肌萎缩症 (SMA) 的潜力。 | |||
T4641 |
Branaplam
LMI 070,NVS-SM1 |
Others; Potassium Channel; DNA/RNA Synthesis | Cell Cycle/Checkpoint; DNA Damage/DNA Repair; Membrane transporter/Ion channel; Others |
Branaplam (LMI 070) 是一种高效、选择性和口服活性的小分子 SMN2 剪接调节剂,对 SMN 的 EC50为 20 nM。它可提高全长 SMN 蛋白并延长其生存期,也抑制 hERG,IC50为 6.3 μM。 | |||
T34664 |
SMN2-Stablizer-27
SMN2-Stablizer 27,SMN2 Stablizer-27 |
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SMN2-Stablizer-27 is a stablizer of survival of motor neuron 2 (SMN2), and a post-translationally stablizing SMN protein for the treatment of spinal muscular atrophy (SMA). | |||
T16902 |
SMND-309
|
Others | Others |
SMND-309 is a metabolite of salvianolic acid B. It also shows neuroprotective effects in cultured neurons and in permanent middle cerebral artery occlusion rats. | |||
T16757 |
Risdiplam
RO7034067,RG7916 |
DNA/RNA Synthesis | Cell Cycle/Checkpoint; DNA Damage/DNA Repair |
Risdiplam (RO7034067) 是一种中枢和外周分布的 SMN2 前 mRNA 剪接修饰剂,可增加运动神经元存活 (SMN) 蛋白水平。 | |||
T3336 |
Rg3039
PF-06687859 |
Others | Others |
Rg3039 (PF-06687859) 是一种具有口服活性、能够透过血脑屏障的DcpS 抑制剂,其IC50=0.069 nM。 | |||
T33764 |
NVS-SM2
|
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NVS-SM2 is an effective small molecule splicing enhancer of SMN2 with oral activity. NVS-SM2 showed increased exon 7 inclusion and upregulated SMN protein expression in SMA fibroblasts and SMN cells Δ7 5025 mouse myoblasts. | |||
T35594 |
Cuspin-1
|
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The Survival of Motor Neurons (SMN) protein participates in RNA splicing. Decreases in SMN, typically a consequence of defects in the smn1 gene, result in the death of motor neurons and lead to the neurodegenerative disease, spinal muscular atrophy (SMA). Cuspin-1 is a small molecule upregulator of SMN that has been shown in vitro to increase levels of SMN in SMA patient fibroblasts by 50% at 18 μM. Its mechanism of action is thought to involve increased phosphorylation of ERK to initiate Ras-Ra... | |||
T26893 |
Branaplam mesylate (1562338-42-4 free base)
LMI070,Branaplam mesylate,LMI 070,LMI-070 |
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Branaplam is a highly potent, selective and orally active SMN2 splicing modulator. Branaplam increases the amount of functional SMN protein produced by the SMN2 gene through modifying its splicing. | |||
T78046 |
RG7800 tetrahydrochloride
RO6885247 tetrahydrochloride |
DNA/RNA Synthesis | Cell Cycle/Checkpoint; DNA Damage/DNA Repair |
RG7800 hydrochloride为一可口服SMN2剪接调节剂,其对SMN2剪接及SMN蛋白的EC1.5x值分别为23 nM及87 nM。 | |||
T28067 |
ML372
ML 372,ML-372 |
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ML372 is a potent and selective SMN Modulator (EC50 = 12 nM, 325% increase inSMN2) with good potency, pharmacokinetics, tolerance, and CNS penetration. | |||
T62381 | Branaplam hydrochloride | ||
Branaplam (LMI070; NVS-SM1) hydrochloride 是一种选择性的、口服具有活力的 SMN2 拼接调节剂,能够作用于 SMN (EC50: 20 nM)。Branaplam hydrochloride 能够抑制 hERG (IC50: 6.3 μM)。在严重的脊髓性肌萎缩症 (SMA) 小鼠模型中,Branaplam hydrochloride 能够增加全长 SMN 蛋白并提高其生存期。 | |||
T64288 | RG7800 hydrochloride | ||
RG7800 hydrochloride 是一种口服具有活力的 SMN2 剪接调节剂,能够作用于 SMN2 剪接 (EC1.5x: 23 nM) 及 SMN 蛋白 (EC1.5x: 87 nM)。 | |||
T38656 | Nusinersen | ||
Nusinersen is an antisense oligonucleotide drug that modifies pre–messenger RNA splicing of the SMN2 gene and thus promotes increased production of full-length SMN protein. | |||
T12718 |
RG7800 hydrochloride (1449598-06-4 free base)
RO6885247 hydrochloride,RG7800 hydrochloride |
Others | Others |
RG7800 hydrochloride is an orally active modulator of SMN2 splicing. |
Cat. No. | Product Name | Species | Expression System |
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TMPY-03047 |
Stathmin 1 Protein, Human, Recombinant (His)
SMN,OP18,stathmin 1,LAP18,C1orf215,PP17,Lag,PP19,PR... |
Human | E. coli |
Stathmin1 (STMN1) is a cytosolic phosphoprotein that regulates cellular microtubule dynamics and is known to have oncogenic activity. STMN1 is a possible biomarker for paclitaxel sensitivity and poor prognosis in GC and could be a novel therapeutic target in metastatic GC. STMN1 expression might serve as a biomarker for determining patient atypical meningioma prognosis. Stathmin1 (STMN1) is a cytosolic protein involved in microtubule dynamics through inhibition of tubulin polymerization and prom... | |||
TMPH-02920 |
SMN1 Protein, Mouse, Recombinant (His & Myc)
|
Mouse | HEK293 |
The SMN complex catalyzes the assembly of small nuclear ribonucleoproteins (snRNPs), the building blocks of the spliceosome, and thereby plays an important role in the splicing of cellular pre-mRNAs. Most spliceosomal snRNPs contain a common set of Sm proteins SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE, SNRPF and SNRPG that assemble in a heptameric protein ring on the Sm site of the small nuclear RNA to form the core snRNP (Sm core). In the cytosol, the Sm proteins SNRPD1, SNRPD2, SNRPE, SNRPF and SNR... | |||
TMPH-03209 |
CLNS1A Protein, Rabbit, Recombinant (His & Myc)
I(Cln),Chloride conductance regulatory protein ICln,Methylos... |
Rabbit | E. coli |
Involved in both the assembly of spliceosomal snRNPs and the methylation of Sm proteins. Chaperone that regulates the assembly of spliceosomal U1, U2, U4 and U5 small nuclear ribonucleoproteins (snRNPs), the building blocks of the spliceosome, and thereby plays an important role in the splicing of cellular pre-mRNAs. Most spliceosomal snRNPs contain a common set of Sm proteins SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE, SNRPF and SNRPG that assemble in a heptameric protein ring on the Sm site of the s... | |||
TMPY-03456 |
SRFBP1 Protein, Human, Recombinant (His & GST)
STRAP,BUD22,P49,serum response factor binding protein 1,p49/... |
Human | Baculovirus-Insect Cells |
SRFBP1 contains 7 WD repeats and belongs to the WD repeat STRAP family. SRFBP1 may play a role in the cellular distribution of the SMN complex. The SMN complex plays an essential role in spliceosomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing in the nucleus. SRFBP1 negatively regulates TGF-beta signaling but positively regulates the PDPK1 kinase activity by enhancing its autophosphorylation and by significantly reducing the association of PDPK1 with 14-3-3 protein. SRF... | |||
TMPH-02228 |
TRA2B Protein, Human, Recombinant (His & Myc)
|
Human | Baculovirus |
Sequence-specific RNA-binding protein which participates in the control of pre-mRNA splicing. Can either activate or suppress exon inclusion. Acts additively with RBMX to promote exon 7 inclusion of the survival motor neuron SMN2. Activates the splicing of MAPT/Tau exon 10. Alters pre-mRNA splicing patterns by antagonizing the effects of splicing regulators, like RBMX. Binds to the AG-rich SE2 domain in the SMN exon 7 RNA. Binds to pre-mRNA. | |||
TMPH-02229 |
TRA2B Protein, Human, Recombinant (His & SUMO)
|
Human | E. coli |
Sequence-specific RNA-binding protein which participates in the control of pre-mRNA splicing. Can either activate or suppress exon inclusion. Acts additively with RBMX to promote exon 7 inclusion of the survival motor neuron SMN2. Activates the splicing of MAPT/Tau exon 10. Alters pre-mRNA splicing patterns by antagonizing the effects of splicing regulators, like RBMX. Binds to the AG-rich SE2 domain in the SMN exon 7 RNA. Binds to pre-mRNA. | |||
TMPH-01679 |
WDR77 Protein, Human, Recombinant (His & SUMO)
|
Human | E. coli |
Non-catalytic component of the methylosome complex, composed of PRMT5, WDR77 and CLNS1A, which modifies specific arginines to dimethylarginines in several spliceosomal Sm proteins and histones. This modification targets Sm proteins to the survival of motor neurons (SMN) complex for assembly into small nuclear ribonucleoprotein core particles. Might play a role in transcription regulation. The methylosome complex also methylates the Piwi proteins (PIWIL1, PIWIL2 and PIWIL4), methylation of Piwi p... |