Skip Header

You are using a version of Internet Explorer that may not display all features of this website. Please upgrade to a modern browser.
Contribute Send feedback
Read comments (?) or add your own

P97801 (SMN_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 123. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (2) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Survival motor neuron protein
Gene names
Name:Smn1
Synonyms:Smn
OrganismMus musculus (Mouse) [Reference proteome]
Taxonomic identifier10090 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus

Protein attributes

Sequence length288 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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. May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs). Ref.10

Subunit structure

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, SYNCRIP and with several spliceosomal snRNP core Sm proteins, including SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE and ILF3. Interacts with OSTF1, LSM10, LSM11 and RPP20/POP7. Ref.5

Subcellular location

Cytoplasm By similarity. Nucleusgem By similarity. Cytoplasmic granule By similarity. CytoplasmmyofibrilsarcomereZ line. Note: Under stress conditions colocalizes with Rpp20/Pop7 in punctuated cytoplasmic granules By similarity. Colocalizes with Actn at the Z-line of skeletal muscle. Ref.7

Domain

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

Sequence similarities

Belongs to the SMN family.

Contains 1 Tudor domain.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 288288Survival motor neuron protein
PRO_0000218904

Regions

Domain88 – 14861Tudor
Region10 – 4132P1 (binding site for GEMIN2) By similarity
Region94 – 204111Required for interaction with RPP20/POP7 By similarity
Region235 – 26228P2 (binding site for SNRPB) By similarity
Region274 – 28815Required for interaction with SYNCRIP By similarity
Compositional bias190 – 1967Poly-Pro
Compositional bias212 – 22211Poly-Pro
Compositional bias239 – 2435Poly-Pro

Amino acid modifications

Modified residue51Phosphoserine By similarity
Modified residue221Phosphothreonine By similarity
Modified residue251Phosphoserine Ref.6 Ref.9
Modified residue281Phosphoserine Ref.8 Ref.9

Experimental info

Mutagenesis1311E → K: Loss of interaction with SYNCRIP. Ref.5
Mutagenesis2571S → I: Loss of interaction with SYNCRIP. Ref.5
Mutagenesis2671Y → C: Loss of interaction with SYNCRIP. Ref.5
Mutagenesis2691T → I: Loss of interaction with SYNCRIP. Ref.5
Mutagenesis2741G → V: Loss of interaction with SYNCRIP. Ref.5

Sequences

Sequence LengthMass (Da)Tools
P97801 [UniParc].

Last modified May 1, 1997. Version 1.
Checksum: 757B3074649F7458

FASTA28831,254
        10         20         30         40         50         60 
MAMGSGGAGS EQEDTVLFRR GTGQSDDSDI WDDTALIKAY DKAVASFKHA LKNGDICETP 

        70         80         90        100        110        120 
DKPKGTARRK PAKKNKSQKK NATTPLKQWK VGDKCSAVWS EDGCIYPATI TSIDFKRETC 

       130        140        150        160        170        180 
VVVYTGYGNR EEQNLSDLLS PTCEVANSTE QNTQENESQV STDDSEHSSR SLRSKAHSKS 

       190        200        210        220        230        240 
KAAPWTSFLP PPPPMPGSGL GPGKPGLKFN GPPPPPPLPP PPFLPCWMPP FPSGPPIIPP 

       250        260        270        280 
PPPISPDCLD DTDALGSMLI SWYMSGYHTG YYMGFRQNKK EGKCSHTN 

« Hide

References

« Hide 'large scale' references
[1]"cDNA isolation, expression, and chromosomal localization of the mouse survival motor neuron gene (Smn)."
Viollet L., Bertrandy S., Brunialti A.L.B., Lefebvre S., Burlet P., Clermont O., Cruaud C., Guenet J.-L., Munnich A., Melki J.
Genomics 40:185-188(1997) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[2]"Cloning, characterization, and copy number of the murine survival motor neuron gene: homolog of the spinal muscular atrophy-determining gene."
Didonato C.J., Chen X.N., Noya D., Korenberg J.R., Nadeau J.H., Simard L.R.
Genome Res. 7:339-352(1997) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[3]"Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death in early mouse embryos."
Schrank B., Goetz R., Gunnersen J.M., Ure J.M., Toyka K.V., Smith A.G., Sendtner M.
Proc. Natl. Acad. Sci. U.S.A. 94:9920-9925(1997) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
Strain: BALB/c.
Tissue: Brain.
[4]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
Strain: FVB/N-3.
Tissue: Mammary gland.
[5]"Specific interaction of Smn, the spinal muscular atrophy determining gene product, with hnRNP-R and gry-rbp/hnRNP-Q: a role for Smn in RNA processing in motor axons?"
Rossoll W., Kroening A.-K., Ohndorf U.-M., Steegborn C., Jablonka S., Sendtner M.
Hum. Mol. Genet. 11:93-105(2002) [PubMed] [Europe PMC] [Abstract]
Cited for: INTERACTION WITH SYNCRIP, MUTAGENESIS OF GLU-131; SER-257; TYR-267; THR-269 AND GLY-274.
[6]"Phosphoproteomic analysis of the developing mouse brain."
Ballif B.A., Villen J., Beausoleil S.A., Schwartz D., Gygi S.P.
Mol. Cell. Proteomics 3:1093-1101(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-25, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
Tissue: Embryonic brain.
[7]"A Drosophila melanogaster model of spinal muscular atrophy reveals a function for SMN in striated muscle."
Rajendra T.K., Gonsalvez G.B., Walker M.P., Shpargel K.B., Salz H.K., Matera A.G.
J. Cell Biol. 176:831-841(2007) [PubMed] [Europe PMC] [Abstract]
Cited for: SUBCELLULAR LOCATION.
[8]"Large-scale phosphorylation analysis of mouse liver."
Villen J., Beausoleil S.A., Gerber S.A., Gygi S.P.
Proc. Natl. Acad. Sci. U.S.A. 104:1488-1493(2007) [PubMed] [Europe PMC] [Abstract]
Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-28, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
Tissue: Liver.
[9]"The phagosomal proteome in interferon-gamma-activated macrophages."
Trost M., English L., Lemieux S., Courcelles M., Desjardins M., Thibault P.
Immunity 30:143-154(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-25 AND SER-28, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[10]"An SMN-dependent U12 splicing event essential for motor circuit function."
Lotti F., Imlach W.L., Saieva L., Beck E.S., Hao le T., Li D.K., Jiao W., Mentis G.Z., Beattie C.E., McCabe B.D., Pellizzoni L.
Cell 151:440-454(2012) [PubMed] [Europe PMC] [Abstract]
Cited for: FUNCTION IN U12 INTRONS SPLICING.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
U63294 mRNA. Translation: AAC53057.1.
U77714 mRNA. Translation: AAC53144.1.
Y12835 mRNA. Translation: CAA73356.1.
BC045158 mRNA. Translation: AAH45158.1.
RefSeqNP_001239558.1. NM_001252629.1.
NP_035550.1. NM_011420.2.
UniGeneMm.2025.

3D structure databases

ProteinModelPortalP97801.
SMRP97801. Positions 23-48, 87-141, 247-276.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid203344. 7 interactions.
IntActP97801. 3 interactions.
STRING10090.ENSMUSP00000022147.

PTM databases

PhosphoSiteP97801.

Proteomic databases

PaxDbP97801.
PRIDEP97801.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENSMUST00000022147; ENSMUSP00000022147; ENSMUSG00000021645.
GeneID20595.
KEGGmmu:20595.
UCSCuc007rqh.1. mouse.

Organism-specific databases

CTD6606.
MGIMGI:109257. Smn1.

Phylogenomic databases

eggNOGNOG296671.
HOGENOMHOG000232199.
HOVERGENHBG000211.
InParanoidP97801.
KOK13129.
OMATTPLKQW.
OrthoDBEOG7K6PVX.
PhylomeDBP97801.
TreeFamTF318390.

Gene expression databases

ArrayExpressP97801.
BgeeP97801.
CleanExMM_SMN1.
GenevestigatorP97801.

Family and domain databases

InterProIPR010304. Survival_motor_neuron.
IPR002999. Tudor.
[Graphical view]
PfamPF06003. SMN. 1 hit.
[Graphical view]
SMARTSM00333. TUDOR. 1 hit.
[Graphical view]
PROSITEPS50304. TUDOR. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

NextBio298911.
PROP97801.
SOURCESearch...

Entry information

Entry nameSMN_MOUSE
AccessionPrimary (citable) accession number: P97801
Secondary accession number(s): O09092
Entry history
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: May 1, 1997
Last modified: April 16, 2014
This is version 123 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

SIMILARITY comments

Index of protein domains and families

MGD cross-references

Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot