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P97801

- SMN_MOUSE

UniProt

P97801 - SMN_MOUSE

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Protein

Survival motor neuron protein

Gene

Smn1

Organism
Mus musculus (Mouse)
Status
Reviewed - 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. May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs).1 Publication

GO - Molecular functioni

  1. RNA binding Source: UniProtKB-KW

GO - Biological processi

  1. axonogenesis Source: MGI
  2. microtubule depolymerization Source: MGI
  3. positive regulation of protein import into nucleus Source: UniProtKB
  4. spliceosomal snRNP assembly Source: UniProtKB
Complete GO annotation...

Keywords - Biological processi

mRNA processing, mRNA splicing, Neurogenesis

Keywords - Ligandi

RNA-binding

Enzyme and pathway databases

ReactomeiREACT_206812. 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
ProteomesiUP000000589: Chromosome 13

Organism-specific databases

MGIiMGI:109257. Smn1.

Subcellular locationi

Cytoplasm By similarity. Nucleusgem By similarity. NucleusCajal body By similarity. Cytoplasmic granule By similarity. CytoplasmmyofibrilsarcomereZ line 1 Publication
Note: 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 (By similarity).By similarity

GO - Cellular componenti

  1. Cajal body Source: UniProtKB
  2. cytoplasm Source: MGI
  3. cytosol Source: UniProtKB
  4. Gemini of coiled bodies Source: UniProtKB
  5. nucleoplasm Source: UniProtKB
  6. nucleus Source: MGI
  7. SMN complex Source: UniProtKB
  8. SMN-Sm protein complex Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Nucleus

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Mutagenesisi131 – 1311E → K: Loss of interaction with SYNCRIP. 1 Publication
Mutagenesisi257 – 2571S → I: Loss of interaction with SYNCRIP. 1 Publication
Mutagenesisi267 – 2671Y → C: Loss of interaction with SYNCRIP. 1 Publication
Mutagenesisi269 – 2691T → I: Loss of interaction with SYNCRIP. 1 Publication
Mutagenesisi274 – 2741G → V: Loss of interaction with SYNCRIP. 1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 288288Survival motor neuron proteinPRO_0000218904Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei5 – 51PhosphoserineBy similarity
Modified residuei22 – 221PhosphothreonineBy similarity
Modified residuei25 – 251Phosphoserine2 Publications
Modified residuei28 – 281Phosphoserine2 Publications

Keywords - PTMi

Phosphoprotein

Proteomic databases

MaxQBiP97801.
PaxDbiP97801.
PRIDEiP97801.

PTM databases

PhosphoSiteiP97801.

Expressioni

Gene expression databases

BgeeiP97801.
CleanExiMM_SMN1.
ExpressionAtlasiP97801. baseline and differential.
GenevestigatoriP97801.

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, 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. Interacts (via C-terminal region) with ZPR1 (via C-terminal region). Interacts (via Tudor domain) with COIL.1 Publication

Binary interactionsi

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

Protein-protein interaction databases

BioGridi203344. 7 interactions.
IntActiP97801. 4 interactions.
STRINGi10090.ENSMUSP00000022147.

Structurei

3D structure databases

ProteinModelPortaliP97801.
SMRiP97801. Positions 23-48, 87-141, 247-276.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini88 – 14861TudorPROSITE-ProRule annotationAdd
BLAST

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni10 – 4132P1 (binding site for GEMIN2)By similarityAdd
BLAST
Regioni94 – 204111Required for interaction with RPP20/POP7By similarityAdd
BLAST
Regioni235 – 26228P2 (binding site for SNRPB)By similarityAdd
BLAST
Regioni274 – 28815Required for interaction with SYNCRIPBy similarityAdd
BLAST

Compositional bias

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Compositional biasi190 – 1967Poly-Pro
Compositional biasi212 – 22211Poly-ProAdd
BLAST
Compositional biasi239 – 2435Poly-Pro

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

eggNOGiNOG296671.
HOGENOMiHOG000232199.
HOVERGENiHBG000211.
InParanoidiP97801.
KOiK13129.
OMAiTTPLKQW.
OrthoDBiEOG7K6PVX.
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:
EMBL
GenBank
DDBJ
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:
EMBL
GenBank
DDBJ
Links Updated
U63294 mRNA. Translation: AAC53057.1 .
U77714 mRNA. Translation: AAC53144.1 .
Y12835 mRNA. Translation: CAA73356.1 .
BC045158 mRNA. Translation: AAH45158.1 .
CCDSi CCDS26725.1.
RefSeqi NP_001239558.1. NM_001252629.1.
NP_035550.1. NM_011420.2.
UniGenei Mm.2025.

3D structure databases

ProteinModelPortali P97801.
SMRi P97801. Positions 23-48, 87-141, 247-276.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 203344. 7 interactions.
IntActi P97801. 4 interactions.
STRINGi 10090.ENSMUSP00000022147.

PTM databases

PhosphoSitei P97801.

Proteomic databases

MaxQBi P97801.
PaxDbi P97801.
PRIDEi P97801.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSMUST00000022147 ; ENSMUSP00000022147 ; ENSMUSG00000021645 .
GeneIDi 20595.
KEGGi mmu:20595.
UCSCi uc007rqh.1. mouse.

Organism-specific databases

CTDi 6606.
MGIi MGI:109257. Smn1.

Phylogenomic databases

eggNOGi NOG296671.
HOGENOMi HOG000232199.
HOVERGENi HBG000211.
InParanoidi P97801.
KOi K13129.
OMAi TTPLKQW.
OrthoDBi EOG7K6PVX.
PhylomeDBi P97801.
TreeFami TF318390.

Enzyme and pathway databases

Reactomei REACT_206812. snRNP Assembly.

Miscellaneous databases

NextBioi 298911.
PROi P97801.
SOURCEi Search...

Gene expression databases

Bgeei P97801.
CleanExi MM_SMN1.
ExpressionAtlasi P97801. baseline and differential.
Genevestigatori P97801.

Family and domain databases

InterProi IPR010304. Survival_motor_neuron.
IPR002999. Tudor.
[Graphical view ]
Pfami PF06003. SMN. 1 hit.
[Graphical view ]
SMARTi SM00333. TUDOR. 1 hit.
[Graphical view ]
PROSITEi PS50304. TUDOR. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  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. 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. 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.

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: October 29, 2014
This is version 129 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

External Data

Dasty 3