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Protein

Survival motor neuron protein

Gene

SMN1

Organism
Bos taurus (Bovine)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at transcript 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

GO - Biological processi

Keywordsi

Molecular functionRNA-binding
Biological processmRNA processing, mRNA splicing, Neurogenesis

Enzyme and pathway databases

ReactomeiR-BTA-191859. snRNP Assembly.

Names & Taxonomyi

Protein namesi
Recommended name:
Survival motor neuron protein
Gene namesi
Name:SMN1
Synonyms:SMN
OrganismiBos taurus (Bovine)
Taxonomic identifieri9913 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCetartiodactylaRuminantiaPecoraBovidaeBovinaeBos
Proteomesi
  • UP000009136 Componenti: Chromosome 20

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 By similarity

  • Note: Colocalizes with actin and at the Z-line of skeletal muscle. 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

GO - Cellular componenti

Keywords - Cellular componenti

Cell projection, Cytoplasm, Nucleus

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002189011 – 287Survival motor neuron proteinAdd BLAST287

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei21PhosphothreonineBy similarity1
Modified residuei24PhosphoserineBy similarity1
Modified residuei27PhosphoserineBy similarity1
Modified residuei65PhosphothreonineBy similarity1
Modified residuei80Phosphothreonine; by PKABy similarity1

Keywords - PTMi

Phosphoprotein

Proteomic databases

PaxDbiO18870.
PRIDEiO18870.

Expressioni

Gene expression databases

BgeeiENSBTAG00000005743.

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. Interacts with SYNCRIP. Interacts (via Tudor domain) with SF3B2 (methylated form) (By similarity).By similarity

Protein-protein interaction databases

STRINGi9913.ENSBTAP00000007547.

Structurei

3D structure databases

ProteinModelPortaliO18870.
SMRiO18870.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini86 – 146TudorPROSITE-ProRule annotationAdd BLAST61

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni9 – 40P1 (binding site for GEMIN2)By similarityAdd BLAST32
Regioni92 – 204Required for interaction with RPP20/POP7By similarityAdd BLAST113
Regioni234 – 261P2 (binding site for SM B)By similarityAdd BLAST28
Regioni273 – 287Required for interaction with SYNCRIPBy similarityAdd BLAST15

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi2 – 7Poly-Gly6
Compositional biasi190 – 193Poly-Pro4
Compositional biasi212 – 220Poly-Pro9
Compositional biasi238 – 242Poly-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

Phylogenomic databases

eggNOGiKOG4327. Eukaryota.
ENOG4111ME8. LUCA.
GeneTreeiENSGT00730000111041.
HOVERGENiHBG000211.
InParanoidiO18870.
KOiK13129.
OMAiAAPWNSF.
OrthoDBiEOG091G0OJK.
TreeFamiTF318390.

Family and domain databases

InterProiView protein in InterPro
IPR010304. Survival_motor_neuron.
IPR002999. Tudor.
PfamiView protein in Pfam
PF06003. SMN. 1 hit.
SMARTiView protein in SMART
SM00333. TUDOR. 1 hit.
PROSITEiView protein in PROSITE
PS50304. TUDOR. 1 hit.

Sequencei

Sequence statusi: Complete.

O18870-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MGGGGGGFPE PEDSVLFRRG TGESDDSDVW DDTALIKAYD KAVASFKHAL
60 70 80 90 100
KNGDISEASE KPKGTPKRKS AKNKSQRKNT TSPSKQWKVG DNCCAIWSED
110 120 130 140 150
GCIYPATIAS IDFKRETCVV VYTGYGNREE QNLSDLLSPT SEVANIEQNA
160 170 180 190 200
QENENESQIS TDESENSSRS PLNKPNNIRS RAAPWNSFLP PPPHMPRSGL
210 220 230 240 250
GPGKSGLNFS GPPPPPPPPP HFLSRWLPPF PAGPPMIPPP PPICPDSLDD
260 270 280
ADALGSMLIS WYMSGYHTGY YMGFKQSQKE GRYSHFN
Length:287
Mass (Da):31,327
Last modified:May 30, 2000 - v2
Checksum:iB1B7EFFCE2682A78
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti61K → E in AAB80943 (Ref. 2) Curated1
Sequence conflicti64G → A in AAB80943 (Ref. 2) Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF035322 Genomic DNA. Translation: AAC17995.1.
AF035323 mRNA. Translation: AAC63439.1.
AF026810 mRNA. Translation: AAB80943.1.
AF016590 Genomic DNA. Translation: AAC04667.1.
AF034259 Genomic DNA. Translation: AAD01979.1.
RefSeqiNP_783632.1. NM_175701.1.
UniGeneiBt.3994.

Genome annotation databases

EnsembliENSBTAT00000007547; ENSBTAP00000007547; ENSBTAG00000005743.
GeneIDi281492.
KEGGibta:281492.

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.

Entry informationi

Entry nameiSMN_BOVIN
AccessioniPrimary (citable) accession number: O18870
Secondary accession number(s): O46481, O62700, Q9TSJ9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: May 30, 2000
Last modified: July 5, 2017
This is version 117 of the entry and version 2 of the sequence. See 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