Survival motor neuron protein (Component of gems 1) (Gemin-1)
1_MAMSS 6_ GGSGG 11_ GVPEQ 16_ EDSVL 21_ FRRGT 26_ GQSDD 31_ SDIWD 36_ DTALI 41_ KAYDK 46_ AVASF 51_ KHALK 56_ NGDIC 61_ ETSGK 66_ PKTTP 71_ KRKPA 76_ KKNKS 81_ QKKNT 86_ AASLQ 91_ QWKVG 96_ DKCSA 101_ IWSED 106_ GCIYP 111_ ATIAS 116_ IDFKR 121_ ETCVV 126_ VYTGY 131_ GNREE 136_ QNLSD 141_ LLSPI 146_ CEVAN 151_ NIEQN 156_ AQENE 161_ NESQV 166_ STDES 171_ ENSRS 176_ PGNKS 181_ DNIKP 186_ KSAPW 191_ NSFLP 196_ PPPPM 201_ PGPRL 206_ GPGKP 211_ GLKFN 216_ GPPPP 221_ PPPPP 226_ PHLLS 231_ CWLPP 236_ FPSGP 241_ PIIPP 246_ PPPIC 251_ PDSLD 256_ DADAL 261_ GSMLI 266_ SWYMS 271_ GYHTG 276_ YYMGF 281_ RQNQK 286_EGRCS
1: 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 (PubMed:18984161, PubMed:9845364). 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) (PubMed:18984161). 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 (PubMed:18984161). To assemble core snRNPs, the SMN complex accepts the trapped 5Sm proteins from CLNS1A forming an intermediate (PubMed:18984161). Within the SMN complex, SMN1 acts as a structural backbone and together with GEMIN2 it gathers the Sm complex subunits (PubMed:17178713, PubMed:21816274, PubMed:22101937). 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 (PubMed:31799625). Ensures the correct splicing of U12 intron-containing genes that may be important for normal motor and proprioceptive neurons development (PubMed:23063131). 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 (PubMed:26700805). May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs)