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Protein

Survival motor neuron protein

Gene

Smn1

Organism
Rattus norvegicus (Rat)
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 similarity

GO - Molecular functioni

  • fibroblast growth factor binding Source: RGD
  • RNA binding Source: UniProtKB-KW

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

mRNA processing, mRNA splicing, Neurogenesis

Keywords - Ligandi

RNA-binding

Names & Taxonomyi

Protein namesi
Recommended name:
Survival motor neuron protein
Gene namesi
Name:Smn1
Synonyms:Smn
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Unplaced

Organism-specific databases

RGDi620755. Smn1.

Subcellular locationi

  • Cytoplasm By similarity
  • Nucleusgem By similarity
  • NucleusCajal body By similarity
  • Cytoplasmic granule By similarity
  • CytoplasmmyofibrilsarcomereZ line By similarity

  • Note: Under stress conditions colocalizes with RPP20/POP7 in punctuated cytoplasmic granules. Colocalizes with Actn at the Z-line of skeletal muscle. 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

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

Keywords - Cellular componenti

Cytoplasm, Nucleus

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 289289Survival motor neuron proteinPRO_0000218905Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei23 – 231PhosphothreonineCombined sources
Modified residuei26 – 261PhosphoserineCombined sources
Modified residuei29 – 291PhosphoserineCombined sources

Keywords - PTMi

Phosphoprotein

Proteomic databases

PaxDbiO35876.
PRIDEiO35876.

PTM databases

iPTMnetiO35876.
PhosphoSiteiO35876.

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. Interacts with SETX; recruits SETX to POLR2A. Interacts with POLR2A (via the C-terminal domain (CTD)). Interacts with PRMT5. Interacts with XRN2.By similarity

GO - Molecular functioni

  • fibroblast growth factor binding Source: RGD

Protein-protein interaction databases

BioGridi249016. 1 interaction.
IntActiO35876. 1 interaction.
STRINGi10116.ENSRNOP00000024456.

Structurei

3D structure databases

ProteinModelPortaliO35876.
SMRiO35876. Positions 87-145.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini89 – 14961TudorPROSITE-ProRule annotationAdd
BLAST

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni11 – 4232P1 (binding site for GEMIN2)By similarityAdd
BLAST
Regioni95 – 205111Required for interaction with RPP20/POP7By similarityAdd
BLAST
Regioni235 – 26228P2 (binding site for SM B)By similarityAdd
BLAST
Regioni274 – 28916Required for interaction with SYNCRIPBy similarityAdd
BLAST

Compositional bias

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Compositional biasi191 – 1977Poly-Pro
Compositional biasi213 – 22210Poly-Pro
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

eggNOGiKOG4327. Eukaryota.
ENOG4111ME8. LUCA.
HOGENOMiHOG000232199.
HOVERGENiHBG000211.
InParanoidiO35876.
KOiK13129.
PhylomeDBiO35876.

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.

O35876-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAMGSGGGAG SEQEDTVLFR RGTGQSDDSD IWDDTALIKA YDKAVASFKH
60 70 80 90 100
ALKNGDMCET SDKPKGTARR KPAKKNKNQK KNATAPLKQW KAGDKCSAVW
110 120 130 140 150
SEDGCVYPAT ITSVDLKRET CVVVYTGYGN KEEQNLSDLL SPTCEVANNT
160 170 180 190 200
EQNTQENESQ VSTDDSEHSS RSLRSKAHSK SKAAPWTSFL PPPPPVPGAG
210 220 230 240 250
LGPGKPGLRF SGPPPPPPPP PPFLPCWMPP FPSGPPIIPP PPPISPDCLD
260 270 280
DTDALGSMLI SWYMSGYHTG YYMGFRQNKK EGKKCSHTN
Length:289
Mass (Da):31,193
Last modified:January 1, 1998 - v1
Checksum:iA8236F80791CE52B
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti8 – 81Missing in AAC01747 (PubMed:9758161).Curated
Sequence conflicti65 – 651K → E in AAC01747 (PubMed:9758161).Curated
Sequence conflicti211 – 2111S → N in AAC01747 (PubMed:9758161).Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U75369 mRNA. Translation: AAB96377.1.
AF044910 mRNA. Translation: AAC01747.1.
RefSeqiNP_071954.1. NM_022509.1.
UniGeneiRn.1119.

Genome annotation databases

GeneIDi64301.
KEGGirno:64301.
UCSCiRGD:620755. rat.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U75369 mRNA. Translation: AAB96377.1.
AF044910 mRNA. Translation: AAC01747.1.
RefSeqiNP_071954.1. NM_022509.1.
UniGeneiRn.1119.

3D structure databases

ProteinModelPortaliO35876.
SMRiO35876. Positions 87-145.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi249016. 1 interaction.
IntActiO35876. 1 interaction.
STRINGi10116.ENSRNOP00000024456.

PTM databases

iPTMnetiO35876.
PhosphoSiteiO35876.

Proteomic databases

PaxDbiO35876.
PRIDEiO35876.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

GeneIDi64301.
KEGGirno:64301.
UCSCiRGD:620755. rat.

Organism-specific databases

CTDi6606.
RGDi620755. Smn1.

Phylogenomic databases

eggNOGiKOG4327. Eukaryota.
ENOG4111ME8. LUCA.
HOGENOMiHOG000232199.
HOVERGENiHBG000211.
InParanoidiO35876.
KOiK13129.
PhylomeDBiO35876.

Miscellaneous databases

PROiO35876.

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...

Publicationsi

« Hide 'large scale' publications
  1. "Expression of the SMN gene, the spinal muscular atrophy determining gene, in the mammalian central nervous system."
    Battaglia G., Princivalle A., Forti F., Lizier C., Zeviani M.
    Hum. Mol. Genet. 6:1961-1971(1997) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Strain: Sprague-Dawley.
    Tissue: Liver.
  2. "Survival motor neuron (SMN) protein in rat is expressed as different molecular forms and is developmentally regulated."
    La Bella V., Cisterni C., Salaun D., Pettmann B.
    Eur. J. Neurosci. 10:2913-2923(1998) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Strain: Sprague-Dawley.
  3. "Quantitative maps of protein phosphorylation sites across 14 different rat organs and tissues."
    Lundby A., Secher A., Lage K., Nordsborg N.B., Dmytriyev A., Lundby C., Olsen J.V.
    Nat. Commun. 3:876-876(2012) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT THR-23; SER-26 AND SER-29, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

Entry informationi

Entry nameiSMN_RAT
AccessioniPrimary (citable) accession number: O35876
Secondary accession number(s): O55045
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: January 1, 1998
Last modified: June 8, 2016
This is version 121 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. 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.