KEGG   PATHWAY: hsa03040Help
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
BRITE hierarchy
Pathway map
Spliceosome
hsa03040

All organismsOrtholog table
Module
Spliceosome, U1-snRNP [PATH:hsa03040]
Spliceosome, U2-snRNP [PATH:hsa03040]
Spliceosome, Prp19/CDC5L complex [PATH:hsa03040]
Spliceosome, U4/U6.U5 tri-snRNP [PATH:hsa03040]
Spliceosome, 35S U5-snRNP [PATH:hsa03040]
Lsm 2-8 complex [PATH:hsa03040]
Sm core complex [PATH:hsa03040]
Cap binding complex [PATH:hsa03040]
THC complex [PATH:hsa03040]
TREX complex [PATH:hsa03040]
Exon junction complex (EJC) [PATH:hsa03040]
Disease
H00527  
Retinitis pigmentosa (RP)
H01376  
Acrofacial dysostosis
H01481  
Myelodysplastic syndrome
Other DBs
BSID: 
GO: 
Organism
Homo sapiens (human) [GN:hsa]
Gene
26827  
RNU6-1; RNA, U6 small nuclear 1 [KO:K14280]
9879  
DDX46; DEAD-box helicase 46 [KO:K12811] [EC:3.6.4.13]
7919  
DDX39B; DEAD-box helicase 39B [KO:K12812] [EC:3.6.4.13]
8449  
DHX16; DEAH-box helicase 16 [KO:K12813] [EC:3.6.4.13]
9785  
DHX38; DEAH-box helicase 38 [KO:K12815] [EC:3.6.4.13]
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:3.6.4.13]
10569  
SLU7; SLU7 homolog, splicing factor [KO:K12819]
1665  
DHX15; DEAH-box helicase 15 [KO:K12820] [EC:3.6.4.13]
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:3.6.4.13]
10915  
TCERG1; transcription elongation regulator 1 [KO:K12824]
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:3.6.4.13]
7307  
U2AF1; U2 small nuclear RNA auxiliary factor 1 [KO:K12836]
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; retinitis pigmentosa 9 (autosomal dominant) [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 processing factor 4 [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; SNU13 homolog, small nuclear ribonucleoprotein (U4/U6.U5) [KO:K12845]
11017  
SNRNP27; small nuclear ribonucleoprotein U4/U6.U5 subunit 27 [KO:K12846]
10713  
USP39; ubiquitin specific peptidase 39 [KO:K12847]
9092  
SART1; squamous cell carcinoma antigen recognized by T-cells 1 [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:3.6.4.13]
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:3.6.4.13]
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; breast carcinoma amplified sequence 2 [KO:K12861]
5356  
PLRG1; pleiotropic regulator 1 [KO:K12862]
51503  
CWC15; CWC15 spliceosome-associated protein [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]
3306  
HSPA2; heat shock protein family A (Hsp70) member 2 [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]
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:3.6.4.13]
9939  
RBM8A; RNA binding motif protein 8A [KO:K12876]
4116  
MAGOH; mago homolog, exon junction complex core component [KO:K12877]
55110  
MAGOHB; mago homolog B, exon junction complex core component [KO:K12877]
9984  
THOC1; THO complex 1 [KO:K12878]
57187  
THOC2; THO complex 2 [KO:K12879]
84321  
THOC3; THO complex 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]
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 (C1/C2) [KO:K12884]
27316  
RBMX; RNA binding motif protein, X-linked [KO:K12885]
494115  
RBMXL1; RNA binding motif protein, X-linked like 1 [KO:K12885]
27288  
RBMXL2; RNA binding motif protein, X-linked like 2 [KO:K12885]
139804  
RBMXL3; RNA binding motif protein, X-linked like 3 [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]
8683  
SRSF9; serine and arginine rich splicing factor 9 [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]
29896  
TRA2A; transformer 2 alpha homolog [KO:K12897]
6434  
TRA2B; transformer 2 beta homolog (Drosophila) [KO:K12897]
10772  
SRSF10; serine and arginine rich splicing factor 10 [KO:K12900]
Reference
  Authors
Wahl MC, Will CL, Luhrmann R
  Title
The spliceosome: design principles of a dynamic RNP machine.
  Journal
Cell 136:701-18 (2009)
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)
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)
Reference
  Authors
Ritchie DB, Schellenberg MJ, MacMillan AM
  Title
Spliceosome structure: piece by piece.
  Journal
Biochim Biophys Acta 1789:624-33 (2009)
KO pathway
 

DBGET integrated database retrieval system