KEGG   PATHWAY: hsa03040
Entry
hsa03040                    Pathway                                
Name
Spliceosome - Homo sapiens (human)
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
Pathway map
hsa03040  Spliceosome
hsa03040

Drug
D11837  Zotatifin (USAN/INN)
D12345  Obefazimod (USAN)
Other DBs
GO: 0005681
Organism
Homo sapiens (human) [GN:hsa]
Gene
26863  RNVU1-18; RNA, variant U1 small nuclear 18 [KO:K14276]
26870  RNU1-2; RNA, U1 small nuclear 2 [KO:K14276]
6060  RNU1-4; RNA, U1 small nuclear 4 [KO:K14276]
26869  RNU1-3; RNA, U1 small nuclear 3 [KO:K14276]
26871  RNU1-1; RNA, U1 small nuclear 1 [KO:K14276]
26864  RNVU1-7; RNA, variant U1 small nuclear 7 [KO:K14276]
101954273  RNVU1-1; RNA, variant U1 small nuclear 1 [KO:K14276]
101954268  RNVU1-20; RNA, variant U1 small nuclear 20 [KO:K14276]
101954264  RNVU1-4; RNA, variant U1 small nuclear 4 [KO:K14276]
124904613  U1 spliceosomal RNA [KO:K14276]
101447996  RNVU1-8; RNA, variant U1 small nuclear 8 [KO:K14276]
101954266  RNVU1-14; RNA, variant U1 small nuclear 14 [KO:K14276]
101954267  RNVU1-15; RNA, variant U1 small nuclear 15 [KO:K14276]
101954269  RNVU1-17; RNA, variant U1 small nuclear 17 [KO:K14276]
101954272  RNVU1-3; RNA, variant U1 small nuclear 3 [KO:K14276]
101954276  RNVU1-6; RNA, variant U1 small nuclear 6 [KO:K14276]
101954277  RNVU1-19; RNA, variant U1 small nuclear 19 [KO:K14276]
124903120  U1 spliceosomal RNA [KO:K14276]
124904122  U1 spliceosomal RNA [KO:K14276]
124904616  RNVU1-28; RNA, variant U1 small nuclear 28 [KO:K14276]
124904619  RNVU1-31; RNA, variant U1 small nuclear 31 [KO:K14276]
124904621  RNVU1-27; RNA, variant U1 small nuclear 27 [KO:K14276]
124904624  RNVU1-26; RNA, variant U1 small nuclear 26 [KO:K14276]
124904627  RNVU1-30; RNA, variant U1 small nuclear 30 [KO:K14276]
124904631  U1 spliceosomal RNA [KO:K14276]
124904634  U1 spliceosomal RNA [KO:K14276]
124905307  U1 spliceosomal RNA [KO:K14276]
124905321  U1 spliceosomal RNA [KO:K14276]
124905573  U1 spliceosomal RNA [KO:K14276]
124905574  U1 spliceosomal RNA [KO:K14276]
124905809  U1 spliceosomal RNA [KO:K14276]
6063  RNVU1-2A; RNA, variant U1 small nuclear 2A [KO:K14276]
6066  RNU2-1; RNA, U2 small nuclear 1 [KO:K14277]
124900915  U2 spliceosomal RNA [KO:K14277]
124904145  U2 spliceosomal RNA [KO:K14277]
124904135  U2 spliceosomal RNA [KO:K14277]
124904136  U2 spliceosomal RNA [KO:K14277]
124904137  U2 spliceosomal RNA [KO:K14277]
124904138  U2 spliceosomal RNA [KO:K14277]
124904139  U2 spliceosomal RNA [KO:K14277]
124904140  U2 spliceosomal RNA [KO:K14277]
124904141  U2 spliceosomal RNA [KO:K14277]
124904142  U2 spliceosomal RNA [KO:K14277]
124904143  U2 spliceosomal RNA [KO:K14277]
124904144  U2 spliceosomal RNA [KO:K14277]
124901219  U2 spliceosomal RNA [KO:K14277]
124905062  U2 spliceosomal RNA [KO:K14277]
124907964  U2 spliceosomal RNA [KO:K14277]
124904651  U2 spliceosomal RNA [KO:K14277]
124907965  U2 spliceosomal RNA [KO:K14277]
124904146  U2 spliceosomal RNA [KO:K14277]
124907963  U2 spliceosomal RNA [KO:K14277]
124904147  U2 spliceosomal RNA [KO:K14277]
26834  RNU4-2; RNA, U4 small nuclear 2 [KO:K14278]
26835  RNU4-1; RNA, U4 small nuclear 1 [KO:K14278]
124901224  U4 spliceosomal RNA [KO:K14278]
124901863  U4 spliceosomal RNA [KO:K14278]
124900516  U4 spliceosomal RNA [KO:K14278]
26830  RNU5D-1; RNA, U5D small nuclear 1 [KO:K14279]
26831  RNU5A-1; RNA, U5A small nuclear 1 [KO:K14279]
26832  RNU5B-1; RNA, U5B small nuclear 1 [KO:K14279]
26828  RNU5F-1; RNA, U5F small nuclear 1 [KO:K14279]
26829  RNU5E-1; RNA, U5E small nuclear 1 [KO:K14279]
101954278  RNU6-8; RNA, U6 small nuclear 8 [KO:K14280]
101954275  RNU6-7; RNA, U6 small nuclear 7 [KO:K14280]
26827  RNU6-1; RNA, U6 small nuclear 1 [KO:K14280]
124902331  U6 spliceosomal RNA [KO:K14280]
101954271  RNU6-9; RNA, U6 small nuclear 9 [KO:K14280]
124901516  U6 spliceosomal RNA [KO:K14280]
124907961  U6 spliceosomal RNA [KO:K14280]
103625684  RNU6-2; RNA, U6 small nuclear 2 [KO:K14280]
124900632  U6 spliceosomal RNA [KO:K14280]
124904694  U6 spliceosomal RNA [KO:K14280]
124900887  U6 spliceosomal RNA [KO:K14280]
124904727  U6 spliceosomal RNA [KO:K14280]
124903414  U6 spliceosomal RNA [KO:K14280]
124905326  U6 spliceosomal RNA [KO:K14280]
124908124  U6 spliceosomal RNA [KO:K14280]
124905264  U6 spliceosomal RNA [KO:K14280]
124904107  U6 spliceosomal RNA [KO:K14280]
124904986  U6 spliceosomal RNA [KO:K14280]
124900883  U6 spliceosomal RNA [KO:K14280]
124900488  U6 spliceosomal RNA [KO:K14280]
124907960  U6 spliceosomal RNA [KO:K14280]
124904109  U6 spliceosomal RNA [KO:K14280]
124902335  U6 spliceosomal RNA [KO:K14280]
9879  DDX46; DEAD-box helicase 46 [KO:K12811] [EC:5.6.2.7]
7919  DDX39B; DExD-box helicase 39B [KO:K12812] [EC:5.6.2.7]
8449  DHX16; DEAH-box helicase 16 [KO:K12813] [EC:5.6.2.6]
9785  DHX38; DEAH-box helicase 38 [KO:K12815] [EC:5.6.2.6]
51362  CDC40; cell division cycle 40 [KO:K12816]
8559  PRPF18; pre-mRNA processing factor 18 [KO:K12817]
1659  DHX8; DEAH-box helicase 8 [KO:K12818] [EC:5.6.2.6]
10569  SLU7; SLU7 homolog, splicing factor [KO:K12819]
1665  DHX15; DEAH-box helicase 15 [KO:K12820] [EC:5.6.2.6]
6628  SNRPB; small nuclear ribonucleoprotein polypeptides B and B1 [KO:K11086]
6632  SNRPD1; small nuclear ribonucleoprotein D1 polypeptide [KO:K11087]
6633  SNRPD2; small nuclear ribonucleoprotein D2 polypeptide [KO:K11096]
6634  SNRPD3; small nuclear ribonucleoprotein D3 polypeptide [KO:K11088]
6635  SNRPE; small nuclear ribonucleoprotein polypeptide E [KO:K11097]
6636  SNRPF; small nuclear ribonucleoprotein polypeptide F [KO:K11098]
6637  SNRPG; small nuclear ribonucleoprotein polypeptide G [KO:K11099]
6625  SNRNP70; small nuclear ribonucleoprotein U1 subunit 70 [KO:K11093]
6626  SNRPA; small nuclear ribonucleoprotein polypeptide A [KO:K11091]
6631  SNRPC; small nuclear ribonucleoprotein polypeptide C [KO:K11095]
25766  PRPF40B; pre-mRNA processing factor 40 homolog B [KO:K12821]
55660  PRPF40A; pre-mRNA processing factor 40 homolog A [KO:K12821]
58517  RBM25; RNA binding motif protein 25 [KO:K12822]
1655  DDX5; DEAD-box helicase 5 [KO:K12823] [EC:5.6.2.7]
10915  TCERG1; transcription elongation regulator 1 [KO:K12824]
2521  FUS; FUS RNA binding protein [KO:K13098]
6627  SNRPA1; small nuclear ribonucleoprotein polypeptide A' [KO:K11092]
6629  SNRPB2; small nuclear ribonucleoprotein polypeptide B2 [KO:K11094]
10291  SF3A1; splicing factor 3a subunit 1 [KO:K12825]
8175  SF3A2; splicing factor 3a subunit 2 [KO:K12826]
10946  SF3A3; splicing factor 3a subunit 3 [KO:K12827]
23451  SF3B1; splicing factor 3b subunit 1 [KO:K12828]
10992  SF3B2; splicing factor 3b subunit 2 [KO:K12829]
23450  SF3B3; splicing factor 3b subunit 3 [KO:K12830]
10262  SF3B4; splicing factor 3b subunit 4 [KO:K12831]
83443  SF3B5; splicing factor 3b subunit 5 [KO:K12832]
51639  SF3B6; splicing factor 3b subunit 6 [KO:K12833]
84844  PHF5A; PHD finger protein 5A [KO:K12834]
11325  DDX42; DEAD-box helicase 42 [KO:K12835] [EC:5.6.2.7]
7307  U2AF1; U2 small nuclear RNA auxiliary factor 1 [KO:K12836]
102724594  U2AF1L5; U2 small nuclear RNA auxiliary factor 1 like 5 [KO:K12836]
199746  U2AF1L4; U2 small nuclear RNA auxiliary factor 1 like 4 [KO:K12836]
11338  U2AF2; U2 small nuclear RNA auxiliary factor 2 [KO:K12837]
22827  PUF60; poly(U) binding splicing factor 60 [KO:K12838]
10285  SMNDC1; survival motor neuron domain containing 1 [KO:K12839]
84991  RBM17; RNA binding motif protein 17 [KO:K12840]
10523  CHERP; calcium homeostasis endoplasmic reticulum protein [KO:K12841]
23350  U2SURP; U2 snRNP associated SURP domain containing [KO:K12842]
6100  RP9; RP9 pre-mRNA splicing factor [KO:K19604]
57819  LSM2; LSM2 homolog, U6 small nuclear RNA and mRNA degradation associated [KO:K12621]
27258  LSM3; LSM3 homolog, U6 small nuclear RNA and mRNA degradation associated [KO:K12622]
25804  LSM4; LSM4 homolog, U6 small nuclear RNA and mRNA degradation associated [KO:K12623]
23658  LSM5; LSM5 homolog, U6 small nuclear RNA and mRNA degradation associated [KO:K12624]
11157  LSM6; LSM6 homolog, U6 small nuclear RNA and mRNA degradation associated [KO:K12625]
51690  LSM7; LSM7 homolog, U6 small nuclear RNA and mRNA degradation associated [KO:K12626]
51691  LSM8; LSM8 homolog, U6 small nuclear RNA associated [KO:K12627]
9129  PRPF3; pre-mRNA processing factor 3 [KO:K12843]
9128  PRPF4; pre-mRNA splicing tri-snRNP complex factor PRPF4 [KO:K12662]
10465  PPIH; peptidylprolyl isomerase H [KO:K09567] [EC:5.2.1.8]
26121  PRPF31; pre-mRNA processing factor 31 [KO:K12844]
4809  SNU13; small nuclear ribonucleoprotein 13 [KO:K12845]
11017  SNRNP27; small nuclear ribonucleoprotein U4/U6.U5 subunit 27 [KO:K12846]
10713  USP39; ubiquitin specific peptidase 39 [KO:K12847]
9092  SART1; spliceosome associated factor 1, recruiter of U4/U6.U5 tri-snRNP [KO:K11984]
153527  ZMAT2; zinc finger matrin-type 2 [KO:K12848]
84950  PRPF38A; pre-mRNA processing factor 38A [KO:K12849]
55119  PRPF38B; pre-mRNA processing factor 38B [KO:K12850]
9343  EFTUD2; elongation factor Tu GTP binding domain containing 2 [KO:K12852]
23020  SNRNP200; small nuclear ribonucleoprotein U5 subunit 200 [KO:K12854] [EC:5.6.2.6]
24148  PRPF6; pre-mRNA processing factor 6 [KO:K12855]
10594  PRPF8; pre-mRNA processing factor 8 [KO:K12856]
9410  SNRNP40; small nuclear ribonucleoprotein U5 subunit 40 [KO:K12857]
9416  DDX23; DEAD-box helicase 23 [KO:K12858] [EC:5.6.2.7]
10907  TXNL4A; thioredoxin like 4A [KO:K12859]
27339  PRPF19; pre-mRNA processing factor 19 [KO:K10599] [EC:2.3.2.27]
988  CDC5L; cell division cycle 5 like [KO:K12860]
10286  BCAS2; BCAS2 pre-mRNA processing factor [KO:K12861]
5356  PLRG1; pleiotropic regulator 1 [KO:K12862]
51503  CWC15; CWC15 spliceosome associated protein homolog [KO:K12863]
56259  CTNNBL1; catenin beta like 1 [KO:K12864]
3312  HSPA8; heat shock protein family A (Hsp70) member 8 [KO:K03283]
3303  HSPA1A; heat shock protein family A (Hsp70) member 1A [KO:K03283]
3305  HSPA1L; heat shock protein family A (Hsp70) member 1 like [KO:K03283]
3304  HSPA1B; heat shock protein family A (Hsp70) member 1B [KO:K03283]
3310  HSPA6; heat shock protein family A (Hsp70) member 6 [KO:K03283]
10084  PQBP1; polyglutamine binding protein 1 [KO:K12865]
51729  WBP11; WW domain binding protein 11 [KO:K12866]
22938  SNW1; SNW domain containing 1 [KO:K06063]
56949  XAB2; XPA binding protein 2 [KO:K12867]
25949  SYF2; SYF2 pre-mRNA splicing factor [KO:K12868]
51340  CRNKL1; crooked neck pre-mRNA splicing factor 1 [KO:K12869]
57461  ISY1; ISY1 splicing factor homolog [KO:K12870]
100534599  ISY1-RAB43; ISY1-RAB43 readthrough [KO:K12870]
51645  PPIL1; peptidylprolyl isomerase like 1 [KO:K12733] [EC:5.2.1.8]
10450  PPIE; peptidylprolyl isomerase E [KO:K09564] [EC:5.2.1.8]
151903  CCDC12; coiled-coil domain containing 12 [KO:K12871]
55696  RBM22; RNA binding motif protein 22 [KO:K12872]
8896  BUD31; BUD31 homolog [KO:K12873]
9716  AQR; aquarius intron-binding spliceosomal factor [KO:K12874]
22985  ACIN1; apoptotic chromatin condensation inducer 1 [KO:K12875]
9775  EIF4A3; eukaryotic translation initiation factor 4A3 [KO:K13025] [EC:5.6.2.7]
9939  RBM8A; RNA binding motif protein 8A [KO:K12876]
4116  MAGOH; mago homolog, exon junction complex subunit [KO:K12877]
55110  MAGOHB; mago homolog B, exon junction complex subunit [KO:K12877]
9984  THOC1; THO complex subunit 1 [KO:K12878]
57187  THOC2; THO complex subunit 2 [KO:K12879]
84321  THOC3; THO complex subunit 3 [KO:K12880]
10189  ALYREF; Aly/REF export factor [KO:K12881]
4686  NCBP1; nuclear cap binding protein subunit 1 [KO:K12882]
22916  NCBP2; nuclear cap binding protein subunit 2 [KO:K12883]
392517  NCBP2L; nuclear cap binding protein subunit 2 like [KO:K12883]
220988  HNRNPA3; heterogeneous nuclear ribonucleoprotein A3 [KO:K12741]
3178  HNRNPA1; heterogeneous nuclear ribonucleoprotein A1 [KO:K12741]
144983  HNRNPA1L2; heterogeneous nuclear ribonucleoprotein A1 like 2 [KO:K12741]
3183  HNRNPC; heterogeneous nuclear ribonucleoprotein C [KO:K12884]
27316  RBMX; RNA binding motif protein X-linked [KO:K12885]
3190  HNRNPK; heterogeneous nuclear ribonucleoprotein K [KO:K12886]
4670  HNRNPM; heterogeneous nuclear ribonucleoprotein M [KO:K12887]
3192  HNRNPU; heterogeneous nuclear ribonucleoprotein U [KO:K12888]
5093  PCBP1; poly(rC) binding protein 1 [KO:K12889]
6426  SRSF1; serine and arginine rich splicing factor 1 [KO:K12890]
6427  SRSF2; serine and arginine rich splicing factor 2 [KO:K12891]
10929  SRSF8; serine and arginine rich splicing factor 8 [KO:K12891]
6428  SRSF3; serine and arginine rich splicing factor 3 [KO:K12892]
6429  SRSF4; serine and arginine rich splicing factor 4 [KO:K12893]
6430  SRSF5; serine and arginine rich splicing factor 5 [KO:K12893]
6431  SRSF6; serine and arginine rich splicing factor 6 [KO:K12893]
6432  SRSF7; serine and arginine rich splicing factor 7 [KO:K12896]
8683  SRSF9; serine and arginine rich splicing factor 9 [KO:K21123]
29896  TRA2A; transformer 2 alpha homolog [KO:K12897]
6434  TRA2B; transformer 2 beta homolog [KO:K12897]
10772  SRSF10; serine and arginine rich splicing factor 10 [KO:K12900]
Reference
PMID:19239890
  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
PMID:10978320
  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
PMID:17537823
  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
PMID:19733268
  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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Genome (1)   
   KEGG GENOME (1)   
Gene (217)   
   KEGG GENES (217)   
All databases (218)   

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