KEGG   PATHWAY: ko03040Help
Entry
ko03040                     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
Genetic Information Processing; Transcription
BRITE hierarchy
Pathway map
ko03040  Spliceosome
ko03040

Ortholog table
Module
M00351  Spliceosome, U1-snRNP [PATH:ko03040]
M00352  Spliceosome, U2-snRNP [PATH:ko03040]
M00353  Spliceosome, Prp19/CDC5L complex [PATH:ko03040]
M00354  Spliceosome, U4/U6.U5 tri-snRNP [PATH:ko03040]
M00355  Spliceosome, 35S U5-snRNP [PATH:ko03040]
M00396  Lsm 2-8 complex [PATH:ko03040]
M00398  Sm core complex [PATH:ko03040]
M00399  Cap binding complex [PATH:ko03040]
M00405  THC complex [PATH:ko03040]
M00406  TREX complex [PATH:ko03040]
M00430  Exon junction complex (EJC) [PATH:ko03040]
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
BSID: 124832
GO: 0005681
Orthology
K14276  U1 spliceosomal RNA
K14277  U2 spliceosomal RNA
K14278  U4 spliceosomal RNA
K14279  U5 spliceosomal RNA
K14280  U6 spliceosomal RNA
K12811  ATP-dependent RNA helicase DDX46/PRP5 [EC:3.6.4.13]
K12812  ATP-dependent RNA helicase UAP56/SUB2 [EC:3.6.4.13]
K12813  pre-mRNA-splicing factor ATP-dependent RNA helicase DHX16 [EC:3.6.4.13]
K12814  pre-mRNA-splicing factor ATP-dependent RNA helicase-like protein PRP2 [EC:3.6.4.13]
K12815  pre-mRNA-splicing factor ATP-dependent RNA helicase DHX38/PRP16 [EC:3.6.4.13]
K12816  pre-mRNA-processing factor 17
K12817  pre-mRNA-splicing factor 18
K12818  ATP-dependent RNA helicase DHX8/PRP22 [EC:3.6.4.13]
K12819  pre-mRNA-processing factor SLU7
K12820  pre-mRNA-splicing factor ATP-dependent RNA helicase DHX15/PRP43 [EC:3.6.4.13]
K11086  small nuclear ribonucleoprotein B and B'
K11087  small nuclear ribonucleoprotein D1
K11096  small nuclear ribonucleoprotein D2
K11088  small nuclear ribonucleoprotein D3
K11097  small nuclear ribonucleoprotein E
K11098  small nuclear ribonucleoprotein F
K11099  small nuclear ribonucleoprotein G
K11093  U1 small nuclear ribonucleoprotein 70kDa
K11091  U1 small nuclear ribonucleoprotein A
K11095  U1 small nuclear ribonucleoprotein C
K12821  pre-mRNA-processing factor 40
K12822  RNA-binding protein 25
K12823  ATP-dependent RNA helicase DDX5/DBP2 [EC:3.6.4.13]
K12824  transcription elongation regulator 1
K11092  U2 small nuclear ribonucleoprotein A'
K11094  U2 small nuclear ribonucleoprotein B''
K12825  splicing factor 3A subunit 1
K12826  splicing factor 3A subunit 2
K12827  splicing factor 3A subunit 3
K12828  splicing factor 3B subunit 1
K12829  splicing factor 3B subunit 2
K12830  splicing factor 3B subunit 3
K12831  splicing factor 3B subunit 4
K12832  splicing factor 3B subunit 5
K12833  pre-mRNA branch site protein p14
K12834  PHD finger-like domain-containing protein 5A
K12835  ATP-dependent RNA helicase DDX42 [EC:3.6.4.13]
K12836  splicing factor U2AF 35 kDa subunit
K12837  splicing factor U2AF 65 kDa subunit
K12838  poly(U)-binding-splicing factor PUF60
K12839  survival of motor neuron-related-splicing factor 30
K12840  splicing factor 45
K12841  calcium homeostasis endoplasmic reticulum protein
K12842  U2-associated protein SR140
K19604  retinitis pigmentosa 9 protein
K12621  U6 snRNA-associated Sm-like protein LSm2
K12622  U6 snRNA-associated Sm-like protein LSm3
K12623  U6 snRNA-associated Sm-like protein LSm4
K12624  U6 snRNA-associated Sm-like protein LSm5
K12625  U6 snRNA-associated Sm-like protein LSm6
K12626  U6 snRNA-associated Sm-like protein LSm7
K12627  U6 snRNA-associated Sm-like protein LSm8
K12843  U4/U6 small nuclear ribonucleoprotein PRP3
K12662  U4/U6 small nuclear ribonucleoprotein PRP4
K09567  peptidyl-prolyl isomerase H (cyclophilin H) [EC:5.2.1.8]
K12844  U4/U6 small nuclear ribonucleoprotein PRP31
K12845  U4/U6 small nuclear ribonucleoprotein SNU13
K12846  U4/U6.U5 tri-snRNP-associated protein 3
K12847  U4/U6.U5 tri-snRNP-associated protein 2
K11984  U4/U6.U5 tri-snRNP-associated protein 1
K12848  U4/U6.U5 tri-snRNP component SNU23
K12849  pre-mRNA-splicing factor 38A
K12850  pre-mRNA-splicing factor 38B
K12851  pre-mRNA-splicing factor 38
K12852  116 kDa U5 small nuclear ribonucleoprotein component
K12853  114 kDa U5 small nuclear ribonucleoprotein component
K12854  pre-mRNA-splicing helicase BRR2 [EC:3.6.4.13]
K12855  pre-mRNA-processing factor 6
K12856  pre-mRNA-processing factor 8
K12857  Prp8 binding protein
K12858  ATP-dependent RNA helicase DDX23/PRP28 [EC:3.6.4.13]
K12859  U5 snRNP protein, DIM1 family
K10599  pre-mRNA-processing factor 19 [EC:2.3.2.27]
K12860  pre-mRNA-splicing factor CDC5/CEF1
K12861  pre-mRNA-splicing factor SPF27
K12862  pleiotropic regulator 1
K12863  protein CWC15
K12864  beta-catenin-like protein 1
K03283  heat shock 70kDa protein 1/2/6/8
K12865  polyglutamine-binding protein 1
K12866  WW domain-binding protein 11
K06063  SNW domain-containing protein 1
K12867  pre-mRNA-splicing factor SYF1
K12868  pre-mRNA-splicing factor SYF2
K12869  crooked neck
K12870  pre-mRNA-splicing factor ISY1
K12733  peptidyl-prolyl cis-trans isomerase-like 1 [EC:5.2.1.8]
K09564  peptidyl-prolyl isomerase E (cyclophilin E) [EC:5.2.1.8]
K12871  coiled-coil domain-containing protein 12
K12872  pre-mRNA-splicing factor RBM22/SLT11
K12873  bud site selection protein 31
K12874  intron-binding protein aquarius
K12875  apoptotic chromatin condensation inducer in the nucleus
K13025  ATP-dependent RNA helicase [EC:3.6.4.13]
K12876  RNA-binding protein 8A
K12877  protein mago nashi
K12878  THO complex subunit 1
K12879  THO complex subunit 2
K12880  THO complex subunit 3
K12881  THO complex subunit 4
K12882  nuclear cap-binding protein subunit 1
K12883  nuclear cap-binding protein subunit 2
K12741  heterogeneous nuclear ribonucleoprotein A1/A3
K12884  heterogeneous nuclear ribonucleoprotein C1/C2
K12885  heterogeneous nuclear ribonucleoprotein G
K12886  heterogeneous nuclear ribonucleoprotein K
K12887  heterogeneous nuclear ribonucleoprotein M
K12888  heterogeneous nuclear ribonucleoprotein U
K12889  poly(rC)-binding protein 1
K12890  splicing factor, arginine/serine-rich 1
K12891  splicing factor, arginine/serine-rich 2
K12892  splicing factor, arginine/serine-rich 3
K12893  splicing factor, arginine/serine-rich 4/5/6
K12896  splicing factor, arginine/serine-rich 7
K21123  splicing factor, arginine/serine-rich 9
K12897  transformer-2 protein
K12900  FUS-interacting serine-arginine-rich protein 1
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

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