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Entry
map03040                    Pathway                                

Name
Spliceosome
Description
After transcription, eukaryotic mRNA precursors contain protein-coding exons and noncoding introns. In the following splicing, introns are excised and exons are joined by a macromolecular complex, the spliceosome. The standard spliceosome is made up of five small nuclear ribonucleoproteins (snRNPs), U1, U2, U4, U5, and U6 snRNPs, and several spliceosome-associated proteins (SAPs). Spliceosomes are not a simple stable complex, but a dynamic family of particles that assemble on the mRNA precursor and help fold it into a conformation that allows transesterification to proceed. Various spliceosome forms (e.g. A-, B- and C-complexes) have been identified.
Class
09120 Genetic Information Processing; 09121 Transcription
BRITE hierarchy
Pathway map
map03040  Spliceosome
map03040

Ortholog table
Module
M00351  Spliceosome, U1-snRNP [PATH:map03040]
M00352  Spliceosome, U2-snRNP [PATH:map03040]
M00353  Spliceosome, Prp19/CDC5L complex [PATH:map03040]
M00354  Spliceosome, U4/U6.U5 tri-snRNP [PATH:map03040]
M00355  Spliceosome, 35S U5-snRNP [PATH:map03040]
M00396  Lsm 2-8 complex [PATH:map03040]
M00398  Sm core complex [PATH:map03040]
M00399  Cap binding complex [PATH:map03040]
M00405  THC complex [PATH:map03040]
M00406  TREX complex [PATH:map03040]
M00430  Exon junction complex (EJC) [PATH:map03040]
Disease
H00527  Retinitis pigmentosa (RP)
H01376  Acrofacial dysostosis
H01481  Myelodysplastic syndrome (MDS)
H01838  Mandibulofacial dysostosis with microcephaly
H01839  Burn-McKeown syndrome
H01843  Cerebrocostomandibular syndrome
H01913  Renpenning syndrome
Other DBs
GO: 0005681
Reference
  Authors
Wahl MC, Will CL, Luhrmann R
  Title
The spliceosome: design principles of a dynamic RNP machine.
  Journal
Cell 136:701-18 (2009)
DOI:10.1016/j.cell.2009.02.009
Reference
  Authors
Morris DP, Greenleaf AL
  Title
The splicing factor, Prp40, binds the phosphorylated carboxyl-terminal domain of RNA polymerase II.
  Journal
J Biol Chem 275:39935-43 (2000)
DOI:10.1074/jbc.M004118200
Reference
  Authors
Chen YI, Moore RE, Ge HY, Young MK, Lee TD, Stevens SW
  Title
Proteomic analysis of in vivo-assembled pre-mRNA splicing complexes expands the catalog of participating factors.
  Journal
Nucleic Acids Res 35:3928-44 (2007)
DOI:10.1093/nar/gkm347
Reference
  Authors
Ritchie DB, Schellenberg MJ, MacMillan AM
  Title
Spliceosome structure: piece by piece.
  Journal
Biochim Biophys Acta 1789:624-33 (2009)
DOI:10.1016/j.bbagrm.2009.08.010
KO pathway
ko03040   

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