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SMN complex

Function: 1) role in spliceosomal snRNP assembly in cytoplasm 2) required for pre-mRNA splicing in nucleus 3) H/ACA snoRNPs interact with the SMN complex - this is mediated by interaction between GAR1 & SMN1 or SMN2 - SMN complex may be required for correct assembly of the H/ACA snoRNP complex Structure: - subunits: SMN1, SIP1/GEMIN2, GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8, STRAP/UNRIP Compartment: 1) subnuclear structures next to coiled bodies, called gems, which are highly enriched in spliceosomal snRNPs 2) nucleolus

Related

small nuclear ribonucleoprotein (snRNP) survival motor neuron protein-1; component of gems 1; Gemin-1 (SMN1, SMN, SMNT, SMN2, SMNC)

General

molecular complex

Properties

SUBUNITS: survival motor neuron protein-1 COMPARTMENT: cytoplasm cell nucleus MOTIF: Ser phosphorylation site {S4} Ser phosphorylation site {S5} Ser phosphorylation site {S8} P1 (binding site for SIP1) {13-44} MOTIF: Thr phosphorylation site {T25} Ser phosphorylation site {S28} Ser phosphorylation site {S31} Tudor domain SITE: 91-151 proline-rich region SITE: 195-203 MOTIF: proline residue (SEVERAL) proline-rich region SITE: 217-226 MOTIF: proline residue (SEVERAL) P2 (binding site for SM B) {240-267} MOTIF: proline-rich region SITE: 244-248 MOTIF: proline residue (SEVERAL) SYNCRIP interaction {279-294} survival of motor neuron protein-interacting protein 1 COMPARTMENT: cytoplasm cell nucleus MOTIF: Thr phosphorylation site {T48} glutamine-rich region {101-106} MOTIF: glutamine residue (SEVERAL) Ser phosphorylation site {S166} ATP-dependent RNA helicase DDX20 COMPARTMENT: cytoplasm cell nucleus MOTIF: Ser phosphorylation site {S48} Q motif {62-90} helicase NAME: helicase SITE: 93-264 MOTIF: ATP-binding site NAME: ATP-binding site SITE: 106-113 Ser phosphorylation site {S187} DEAD/H box NAME: DEAD/H box SITE: 211-214 Helicase C-terminal {299-448} SMN interacting {456-548} MOTIF: Ser phosphorylation site {S500} Ser phosphorylation site {S532} Thr phosphorylation site {T552} Ser phosphorylation site {S649} Ser phosphorylation site {S652} Ser phosphorylation site {S654} Ser phosphorylation site {S656} Ser phosphorylation site {S672} Ser phosphorylation site {S677} Ser phosphorylation site {S678} Ser phosphorylation site {S703} Thr phosphorylation site {T705} Ser phosphorylation site {S714} component of gems 4 COMPARTMENT: cytoplasm cell nucleus MOTIF: leucine zipper SITE: 714-735 FOR-BINDING-VIA: leucine zipper Gem-associated protein 5 COMPARTMENT: cytoplasm cell nucleus MOTIF: WD repeat {62-104} WD repeat {107-148} WD repeat {150-189} WD repeat {193-264} WD repeat {280-321} WD repeat {333-374} WD repeat {377-417} WD repeat {424-464} WD repeat {468-509} WD repeat {533-573} WD repeat {576-622} WD repeat {637-677} WD repeat {680-720} lysine-rich region {738-746} MOTIF: lysine residue (SEVERAL) Ser phosphorylation site {S778} Thr phosphorylation site {T807} coiled coil {1362-1393} Gem-associated protein 6 COMPARTMENT: cytoplasm cell nucleus Gem-associated protein 7 COMPARTMENT: cytoplasm cell nucleus Gem-associated protein 8 COMPARTMENT: cytoplasm cell nucleus MOTIF: coiled coil {135-168}

References

UniProt :accession P46977

Components

ATP-dependent RNA helicase DDX20; DEAD box protein 20; DEAD box protein DP 103; component of gems 3; Gemin-3 (DDX20, DP103, GEMIN3) component of gems 4 (Gem-associated protein 4, Gemin-4, p97, GEMIN4) Gem-associated protein 5 (Gemin5) Gem-associated protein 6 (Gemin-6, SIP2, GEMIN6) Gem-associated protein 7 (Gemin-7, SIP3, GEMIN7) Gem-associated protein 8 (protein FAM51A1, GEMIN8, FAM51A1) survival motor neuron protein-1; component of gems 1; Gemin-1 (SMN1, SMN, SMNT, SMN2, SMNC) survival of motor neuron protein-interacting protein 1; SMN-interacting protein 1; component of gems 2; Gemin-2 (SIP1, GEMIN2)