FEATURES

FAMILY

DESCRIPTION

Also known as DDX20_HUMAN, DDX20, DP103, GEMIN3. 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 triggers eviction of the SMN complex, thereby allowing binding of SNRPD3 and SNRPB to complete assembly of the core snRNP. May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs). Part of the core SMN complex that contains SMN1, GEMIN2/SIP1, DDX20/GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8 and STRAP/UNRIP (PubMed:10601333, PubMed:17178713). 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 (PubMed:18984161, PubMed:12095920). Interacts with SMN1; the interaction is direct (PubMed:17178713). Interacts with GEMIN4; the interaction is direct (PubMed:17178713). Interacts with SNUPN; the interaction is direct (PubMed:12095920). Interacts with PPP4R2 (PubMed:12668731). Interacts with FOXL2 (PubMed:16153597). Interacts with EBV EBNA2 and EBNA3C (PubMed:10383418). Interacts with NANOS1 and PUM2 (PubMed:21800163).

Also known as DDX20_HUMAN, DDX20, DP103, GEMIN3. The SMN complex catalyzes the assembly of small nuclear ribonucleoproteins (snRNPs), the building blocks of the spliceosome, and thereby plays an ...

EXTERNAL LINKS

DDX20

Mutations

Mutation(s) distribution

Mutations table