Smn1
entrez
64301
canonical
uniprot
O35876
canonical
ensembl
ENSRNOG00000018067
resolver
ensp
ENSRNOP00000076972
resolver
genesymbol
Smn1
resolver
genesymbol_syn
Smn
resolver
uniprot
O55045
resolver
uniprot
Q6P684
resolver
uniprot_entry
Q6P684_RAT
resolver
uniprot_entry
SMN_RAT
resolver
uniprot_sec
O55045
resolver
amino_acid_sequence
MAMGSGGGAGSEQEDTVLFRRGTGQSDDSDIWDDTALIKAYDKAVASFKHALKNGDMCETSDKPKGTARRKPAKKNKNQKKNATAPLKQWKAGDKCSAVWSEDGCVYPATITSVDLKRETCVVVYTGYGNKEEQNLSDLLSPTCEVANNTEQNTQENESQVSTDDSEHSSRSLRSKAHSKSKAAPWTSFLPPPPPVPGAGLGPGKPGLRFSGPPPPPPPPPPFLPCWMPPFPSGPPIIPPPPPISPDCLDDTDALGSMLISWYMSGYHTGYYMGFRQNKKEGKKCSHTN
uniprot
proteins
function
FUNCTION: The SMN complex catalyzes the assembly of small nuclear ribonucleoproteins (snRNPs), the building blocks of the spliceosome, and 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 (Sm core). 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. To assemble core snRNPs, the SMN complex accepts the trapped 5Sm proteins from CLNS1A forming an intermediate. Binding of snRNA inside 5Sm ultimately triggers eviction of the SMN complex, thereby allowing binding of SNRPD3 and SNRPB to complete assembly of the core snRNP. Within the SMN complex, SMN1 acts as a structural backbone and together with GEMIN2 it gathers the Sm complex subunits. 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). {ECO:0000250|UniProtKB:Q16637}.
uniprot
proteins
mass_dalton
31193
uniprot
proteins
protein_family
SMN family
uniprot
proteins
pubmed
21389246
uniprot
proteins
pubmed
22673903
uniprot
proteins
pubmed
9302277
uniprot
proteins
pubmed
9758161
uniprot
proteins
sequence_length
289
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Nucleus, gem {ECO:0000250|UniProtKB:Q16637}. Nucleus, Cajal body {ECO:0000250|UniProtKB:Q16637}. Cytoplasm {ECO:0000250|UniProtKB:Q16637}. Cytoplasmic granule {ECO:0000250|UniProtKB:Q16637}. Perikaryon {ECO:0000250|UniProtKB:Q16637}. Cell projection, neuron projection {ECO:0000250|UniProtKB:Q16637}. Cell projection, axon {ECO:0000250|UniProtKB:P97801}. Cytoplasm, myofibril, sarcomere, Z line {ECO:0000250|UniProtKB:P97801}. Note=Colocalizes with actin and at the Z-line of skeletal muscle (By similarity). 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). {ECO:0000250|UniProtKB:P97801, ECO:0000250|UniProtKB:Q16637}.
uniprot
proteins