General Information of the Protein
Protein ID
PT04513
Protein Name
Survival motor neuron protein
Secondarily
Protein Name
Component of gems 1
Gemin-1
Gene Name
SMN1
Secondarily
Gene Name
SMN
SMNT
Sequence
MAMSSGGSGGGVPEQEDSVLFRRGTGQSDDSDIWDDTALIKAYDKAVASFKHALKNGDICETSGKPKTTPKRKPAKKNKSQKKNTAASLQQWKVGDKCSAIWSEDGCIYPATIASIDFKRETCVVVYTGYGNREEQNLSDLLSPICEVANNIEQNAQENENESQVSTDESENSRSPGNKSDNIKPKSAPWNSFLPPPPPMPGPRLGPGKPGLKFNGPPPPPPPPPPHLLSCWLPPFPSGPPIIPPPPPICPDSLDDADALGSMLISWYMSGYHTGYYMGFRQNQKEGRCSHSLN
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Organism
Homo sapiens, Human
Protein Classification
Epigenetic regulator
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Reader
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Methyl-lysine/arginine binding protein
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Tudor domain
Function
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 (PubMed:9845364, PubMed:18984161). 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) (PubMed:18984161). In the cytosol, the Sm proteins SNRPD1, SNRPD2, SNRPE, SNRPF and SNRPG are trapped in an inactive 6S pICln-Sm complex by the chaperone CLNS1A that controls the assembly of the core snRNP (PubMed:18984161). To assemble core snRNPs, the SMN complex accepts the trapped 5Sm proteins from CLNS1A forming an intermediate (PubMed:18984161). Within the SMN complex, SMN1 acts as a structural backbone and together with GEMIN2 it gathers the Sm complex subunits (PubMed:21816274, PubMed:22101937, PubMed:17178713). Binding of snRNA inside 5Sm ultimately triggers eviction of the SMN complex, thereby allowing binding of SNRPD3 and SNRPB to complete assembly of the core snRNP (PubMed:31799625). Ensures the correct splicing of U12 intron-containing genes that may be important for normal motor and proprioceptive neurons development (PubMed:23063131). Also required for resolving RNA-DNA hybrids created by RNA polymerase II, that form R-loop in transcription terminal regions, an important step in proper transcription termination (PubMed:26700805). May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs).
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Uniprot ID
Primary ID:
Q16637

Secondarily ID:
A8K0V4
Q13119
Q549U5
Q96J51
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Ensembl ID
ENSG00000172062
HGNC ID
HGNC:11117
Subcellular Location
Nucleus
Gem
Nucleus
Cajal body
Cytoplasm
Cytoplasmic granule
Perikaryon
Cell projection
Neuron projection
Cell projection
Axon
Cytoplasm
Myofibril
Sarcomere
Z line
Map of Molecular Bioactivity Related to the Protein
Map of Molecular Bioactivity Related to the Protein

Protein
Cell Line
Compound

Bioactivity Value:

<= 0.1 μM
> 0.1 μM and <= 10 μM
> 10 μM
Imprecise Activity
Table of Molecular Bioactivities Related to the Protein
Cell Line ID: CL000006 , HEK293
Compound ID Compound Name Compound Formula
CP0417766
N-(1,3-benzothiazol-2-yl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C14H13N3O2S
 1
1
EC50 = 170 nM
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CP0417749
N-(2-fluorophenyl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C13H13FN2O2
 1
1
EC50 = 260 nM
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CP0461849
N-(1H-benzimidazol-2-yl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C14H14N4O2
 1
1
EC50 = 260 nM
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CP0461853
N-phenyl-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C13H14N2O2
 1
1
EC50 = 280 nM
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CP0180642
3-chloro-4-fluoro-N-[5-(1-hydroxycyclobutyl)-1,3-thiazol-2-yl]benzamide
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C14H12ClFN2O2S
 1
1
EC50 = 290 nM
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CP0476133
N-(3-fluorophenyl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C13H13FN2O2
 1
1
EC50 = 340 nM
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CP0210377
5-propan-2-yl-N-pyridin-2-yl-1,2-oxazole-3-carboxamide
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C12H13N3O2
 1
1
EC50 = 360 nM
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CP0454809
N-(3-chloro-4-fluorophenyl)-4-methyl-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C14H14ClFN2O2
 1
1
EC50 = 500 nM
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CP0531613
3-chloro-N-(5-cyclobutyl-1,3-thiazol-2-yl)-4-fluorobenzamide
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C14H12ClFN2OS
 1
1
EC50 = 500 nM
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CP0558692
2,3-dihydroindol-1-yl-(5-propan-2-yl-1,2-oxazol-3-yl)methanone
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C15H16N2O2
 1
1
EC50 = 720 nM
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CP0446922
5-propan-2-yl-N-(1,3-thiazol-2-yl)-1,2-oxazole-3-carboxamide
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C10H11N3O2S
 1
1
EC50 = 780 nM
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CP0285333
MLS000087323
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C13H13ClN2O2
 1
1
EC50 = 860 nM
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CP0447619
N-(4-methylphenyl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C14H16N2O2
 1
1
EC50 = 1400 nM
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CP0476134
N-(3-chloro-4-fluorophenyl)-5-(2-methylpropyl)-1,2-oxazole-3-carboxamide
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C14H14ClFN2O2
 1
1
EC50 = 1500 nM
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CP0531614
3-chloro-4-fluoro-N-(2-propan-2-yl-1,3-thiazol-4-yl)benzamide
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C13H12ClFN2OS
 1
1
EC50 = 1600 nM
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CP0394909
N-(3-chloro-4-fluorophenyl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C13H12ClFN2O2
 1
1
EC50 = 1800 nM
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CP0455772
N-(4-fluorophenyl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C13H13FN2O2
 1
1
EC50 = 1800 nM
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CP0508473
N-(3-methylphenyl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C14H16N2O2
 1
1
EC50 = 1800 nM
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CP0549005
5-tert-butyl-N-(3-chloro-4-fluorophenyl)-1,2-oxazole-3-carboxamide
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C14H14ClFN2O2
 1
1
EC50 = 1930 nM
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CP0417764
N-(1-methylbenzimidazol-2-yl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C15H16N4O2
 1
1
EC50 = 2600 nM
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CP0446920
N-(4-chlorophenyl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C13H13ClN2O2
 1
1
EC50 = 2800 nM
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CP0531615
5-benzyl-N-(3-chloro-4-fluorophenyl)-1,2-oxazole-3-carboxamide
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C17H12ClFN2O2
 1
1
EC50 = 3560 nM
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CP0461841
N-(3-methoxyphenyl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C14H16N2O3
 1
1
EC50 = 3600 nM
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CP0432677
N-(2-methylphenyl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C14H16N2O2
 1
1
EC50 = 5400 nM
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CP0455785
3-chloro-4-fluoro-N-(3-propan-2-yl-1,2-oxazol-5-yl)benzamide
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C13H12ClFN2O2
 1
1
EC50 = 5500 nM
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CP0454808
N-(2-chlorophenyl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C13H13ClN2O2
 1
1
EC50 = 6200 nM
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CP0563525
N-(2-methoxyphenyl)-5-propan-2-yl-1,2-oxazole-3-carboxamide
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C14H16N2O3
 1
1
EC50 = 7300 nM
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CP0446919
N-(3-chloro-4-fluorophenyl)-5-ethyl-1,2-oxazole-3-carboxamide
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C12H10ClFN2O2
 1
1
EC50 = 8800 nM
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CP0454788
3-chloro-4-fluoro-N-(5-propan-2-yl-1,2-oxazol-3-yl)benzamide
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C13H12ClFN2O2
 1
1
EC50 = 9900 nM
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CP0531610
5-isopropyl-N-(3-pyridyl)isoxazole-3-carboxamide
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C12H13N3O2
 1
1
EC50 = 9900 nM
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CP0454758
3-chloro-4-fluoro-N-[(5-propan-2-yl-1,2-oxazol-3-yl)methyl]aniline
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C13H14ClFN2O
 1
1
EC50 = 11200 nM
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CP0534557
N-(3-chloro-4-fluorophenyl)-5-propan-2-yl-1,2-oxazole-3-carbothioamide
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C13H12ClFN2OS
 1
1
EC50 = 13900 nM
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Clinical Information about the Protein
Target 1 ( Survival motor neuron protein (SMN1) )
Target Type Successful Target
Disease 1 Target-related Disease  1
1 Spinal muscular atrophy [ICD-11: 8B61]
Clinical Trial Drug(s) 2 Clinical Trial Drugs  2
1 LMI070 Phase 3
Spinal muscular atrophy
2 RG7800 Phase 1/2
Spinal muscular atrophy
Target 2 ( SMN1 messenger RNA (SMN1 mRNA) )
Target Type Clinical trial Target