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Protein

Survival motor neuron protein

Gene

Smn1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

The SMN complex plays a catalyst role in the assembly of small nuclear ribonucleoproteins (snRNPs), the building blocks of the spliceosome. 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. 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. Dissociation by the SMN complex of CLNS1A from the trapped Sm proteins and their transfer to an SMN-Sm complex triggers the assembly of core snRNPs and their transport to the nucleus. Ensures the correct splicing of U12 intron-containing genes that may be important for normal motor and proprioceptive neurons development. 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. May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs).By similarity1 Publication

GO - Molecular functioni

GO - Biological processi

  • axonogenesis Source: MGI
  • DNA-templated transcription, termination Source: UniProtKB
  • microtubule depolymerization Source: MGI
  • positive regulation of RNA splicing Source: MGI
  • regulation of neuron projection development Source: MGI
  • spliceosomal snRNP assembly Source: UniProtKB
Complete GO annotation...

Keywords - Biological processi

mRNA processing, mRNA splicing, Neurogenesis

Keywords - Ligandi

RNA-binding

Enzyme and pathway databases

ReactomeiR-MMU-191859. snRNP Assembly.

Names & Taxonomyi

Protein namesi
Recommended name:
Survival motor neuron protein
Gene namesi
Name:Smn1
Synonyms:Smn
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 13

Organism-specific databases

MGIiMGI:109257. Smn1.

Subcellular locationi

  • Nucleusgem By similarity
  • NucleusCajal body By similarity
  • Cytoplasm By similarity
  • Cytoplasmic granule By similarity
  • Perikaryon By similarity
  • Cell projection By similarity
  • CytoplasmmyofibrilsarcomereZ line 1 Publication

  • Note: Colocalizes with actin and at the Z-line of skeletal muscle (PubMed:17353360). Under stress conditions colocalizes with RPP20/POP7 in punctuated cytoplasmic granules. Colocalized and redistributed with ZPR1 from the cytoplasm to nuclear gems (Gemini of coiled bodies) and Cajal bodies. Colocalizes with FMR1 in cytoplasmic granules in the soma and neurite cell processes (By similarity).By similarity

GO - Cellular componenti

  • Cajal body Source: UniProtKB
  • cytoplasm Source: MGI
  • cytoplasmic ribonucleoprotein granule Source: UniProtKB
  • cytosol Source: UniProtKB
  • Gemini of coiled bodies Source: UniProtKB
  • neuron projection Source: UniProtKB
  • nucleoplasm Source: UniProtKB
  • nucleus Source: MGI
  • perikaryon Source: UniProtKB
  • SMN complex Source: UniProtKB
  • SMN-Sm protein complex Source: UniProtKB
  • Z disc Source: UniProtKB-SubCell
Complete GO annotation...

Keywords - Cellular componenti

Cell projection, Cytoplasm, Nucleus

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi131E → K: Loss of interaction with SYNCRIP. 1 Publication1
Mutagenesisi257S → I: Loss of interaction with SYNCRIP. 1 Publication1
Mutagenesisi267Y → C: Loss of interaction with SYNCRIP. 1 Publication1
Mutagenesisi269T → I: Loss of interaction with SYNCRIP. 1 Publication1
Mutagenesisi274G → V: Loss of interaction with SYNCRIP. 1 Publication1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002189041 – 288Survival motor neuron proteinAdd BLAST288

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei5PhosphoserineBy similarity1
Modified residuei22PhosphothreonineCombined sources1
Modified residuei25PhosphoserineCombined sources1
Modified residuei28PhosphoserineCombined sources1
Modified residuei66PhosphothreonineBy similarity1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiP97801.
PaxDbiP97801.
PeptideAtlasiP97801.
PRIDEiP97801.

PTM databases

iPTMnetiP97801.
PhosphoSitePlusiP97801.

Expressioni

Gene expression databases

BgeeiENSMUSG00000021645.
CleanExiMM_SMN1.
ExpressionAtlasiP97801. baseline and differential.
GenevisibleiP97801. MM.

Interactioni

Subunit structurei

Homodimer. Part of the core SMN complex that contains SMN1, GEMIN2/SIP1, DDX20/GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8 and STRAP/UNRIP. Part of the SMN-Sm complex that contains SMN1, GEMIN2/SIP1, DDX20/GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8, STRAP/UNRIP and the Sm proteins SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE, SNRPF and SNRPG. Component of an import snRNP complex composed of KPNB1, RNUT1, SMN1 and ZNF259. Interacts with DDX20, FBL, NOLA1, RNUT1 and with several spliceosomal snRNP core Sm proteins, including SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE and ILF3. Interacts with OSTF1, LSM10, LSM11 and RPP20/POP7. Interacts (via C-terminal region) with ZPR1 (via C-terminal region). Interacts (via Tudor domain) with COIL. Interacts with SETX; recruits SETX to POLR2A. Interacts with POLR2A (via the C-terminal domain (CTD)). Interacts with PRMT5. Interacts with XRN2. Interacts (via C-terminus) with FMR1 (via C-terminus); the interaction is direct and occurs in a RNA-independent manner (By similarity). Interacts with SYNCRIP (PubMed:11773003).By similarity1 Publication

Binary interactionsi

WithEntry#Exp.IntActNotes
NS1P031343EBI-309807,EBI-9515229From a different organism.

GO - Molecular functioni

Protein-protein interaction databases

BioGridi203344. 75 interactors.
IntActiP97801. 73 interactors.
STRINGi10090.ENSMUSP00000022147.

Structurei

3D structure databases

ProteinModelPortaliP97801.
SMRiP97801.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini88 – 148TudorPROSITE-ProRule annotationAdd BLAST61

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni10 – 41P1 (binding site for GEMIN2)By similarityAdd BLAST32
Regioni94 – 204Required for interaction with RPP20/POP7By similarityAdd BLAST111
Regioni235 – 262P2 (binding site for SNRPB)By similarityAdd BLAST28
Regioni274 – 288Required for interaction with SYNCRIPBy similarityAdd BLAST15

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi190 – 196Poly-Pro7
Compositional biasi212 – 222Poly-ProAdd BLAST11
Compositional biasi239 – 243Poly-Pro5

Domaini

The Tudor domain mediates association with dimethylarginines, which are common in snRNP proteins.By similarity

Sequence similaritiesi

Belongs to the SMN family.Curated
Contains 1 Tudor domain.PROSITE-ProRule annotation

Phylogenomic databases

eggNOGiKOG4327. Eukaryota.
ENOG4111ME8. LUCA.
GeneTreeiENSGT00730000111041.
HOGENOMiHOG000232199.
HOVERGENiHBG000211.
InParanoidiP97801.
KOiK13129.
OMAiTTPLKQW.
OrthoDBiEOG091G0OJK.
PhylomeDBiP97801.
TreeFamiTF318390.

Family and domain databases

InterProiIPR010304. Survival_motor_neuron.
IPR002999. Tudor.
[Graphical view]
PfamiPF06003. SMN. 1 hit.
[Graphical view]
SMARTiSM00333. TUDOR. 1 hit.
[Graphical view]
PROSITEiPS50304. TUDOR. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

P97801-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAMGSGGAGS EQEDTVLFRR GTGQSDDSDI WDDTALIKAY DKAVASFKHA
60 70 80 90 100
LKNGDICETP DKPKGTARRK PAKKNKSQKK NATTPLKQWK VGDKCSAVWS
110 120 130 140 150
EDGCIYPATI TSIDFKRETC VVVYTGYGNR EEQNLSDLLS PTCEVANSTE
160 170 180 190 200
QNTQENESQV STDDSEHSSR SLRSKAHSKS KAAPWTSFLP PPPPMPGSGL
210 220 230 240 250
GPGKPGLKFN GPPPPPPLPP PPFLPCWMPP FPSGPPIIPP PPPISPDCLD
260 270 280
DTDALGSMLI SWYMSGYHTG YYMGFRQNKK EGKCSHTN
Length:288
Mass (Da):31,254
Last modified:May 1, 1997 - v1
Checksum:i757B3074649F7458
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U63294 mRNA. Translation: AAC53057.1.
U77714 mRNA. Translation: AAC53144.1.
Y12835 mRNA. Translation: CAA73356.1.
BC045158 mRNA. Translation: AAH45158.1.
CCDSiCCDS26725.1.
RefSeqiNP_001239558.1. NM_001252629.1.
NP_035550.1. NM_011420.2.
UniGeneiMm.2025.

Genome annotation databases

EnsembliENSMUST00000022147; ENSMUSP00000022147; ENSMUSG00000021645.
GeneIDi20595.
KEGGimmu:20595.
UCSCiuc007rqh.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U63294 mRNA. Translation: AAC53057.1.
U77714 mRNA. Translation: AAC53144.1.
Y12835 mRNA. Translation: CAA73356.1.
BC045158 mRNA. Translation: AAH45158.1.
CCDSiCCDS26725.1.
RefSeqiNP_001239558.1. NM_001252629.1.
NP_035550.1. NM_011420.2.
UniGeneiMm.2025.

3D structure databases

ProteinModelPortaliP97801.
SMRiP97801.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi203344. 75 interactors.
IntActiP97801. 73 interactors.
STRINGi10090.ENSMUSP00000022147.

PTM databases

iPTMnetiP97801.
PhosphoSitePlusiP97801.

Proteomic databases

EPDiP97801.
PaxDbiP97801.
PeptideAtlasiP97801.
PRIDEiP97801.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000022147; ENSMUSP00000022147; ENSMUSG00000021645.
GeneIDi20595.
KEGGimmu:20595.
UCSCiuc007rqh.1. mouse.

Organism-specific databases

CTDi6606.
MGIiMGI:109257. Smn1.

Phylogenomic databases

eggNOGiKOG4327. Eukaryota.
ENOG4111ME8. LUCA.
GeneTreeiENSGT00730000111041.
HOGENOMiHOG000232199.
HOVERGENiHBG000211.
InParanoidiP97801.
KOiK13129.
OMAiTTPLKQW.
OrthoDBiEOG091G0OJK.
PhylomeDBiP97801.
TreeFamiTF318390.

Enzyme and pathway databases

ReactomeiR-MMU-191859. snRNP Assembly.

Miscellaneous databases

PROiP97801.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000021645.
CleanExiMM_SMN1.
ExpressionAtlasiP97801. baseline and differential.
GenevisibleiP97801. MM.

Family and domain databases

InterProiIPR010304. Survival_motor_neuron.
IPR002999. Tudor.
[Graphical view]
PfamiPF06003. SMN. 1 hit.
[Graphical view]
SMARTiSM00333. TUDOR. 1 hit.
[Graphical view]
PROSITEiPS50304. TUDOR. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiSMN_MOUSE
AccessioniPrimary (citable) accession number: P97801
Secondary accession number(s): O09092
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: May 1, 1997
Last modified: November 30, 2016
This is version 152 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.