KEGG   PATHWAY: hsa03008Help
Entry
hsa03008                    Pathway                                

Name
Ribosome biogenesis in eukaryotes - Homo sapiens (human)
Description
Ribosomes are the cellular factories responsible for making proteins. In eukaryotes, ribosome biogenesis involves the production and correct assembly of four rRNAs and about 80 ribosomal proteins. It requires hundreds of factors not present in the mature particle. In the absence of these proteins, ribosome biogenesis is stalled and cell growth is terminated even under optimal growth conditions. The primary pre-rRNA transcript is  assembled into the 90S pre-ribosome, which contains both 40S and 60S assembly factors. Within this complex, the pre-rRNA is cleaved. pre-60S ribosomes are subjected to several sequential processing steps in the nucleoplasm involving numerous assembly intermediates before it is exported to the cytoplasm and matured into the 60S ribosomal subunit. The pre-40S ribosome is matured to the small ribosomal subunit in the cytoplasm by cleavage.
Class
Genetic Information Processing; Translation
BRITE hierarchy
Pathway map
Ribosome biogenesis in eukaryotes
hsa03008

All organismsOrtholog table
Module
H/ACA ribonucleoprotein complex [PATH:hsa03008]
Disease
H00063  
Spinocerebellar ataxia (SCA)
H00439  
Shwachman-Diamond syndrome (SDS)
H00507  
Dyskeratosis congenita (DC)
H00518  
RMRP related disorders
H00610  
Treacher Collins syndrome
H00612  
Primary open angle glaucoma
H00616  
Bowen-Conradi syndrome (BCS)
H00621  
Alopecia neurologic defects and endocrinopathy syndrome (ANE syndrome)
H00624  
Familial cholestasis
H00788  
Hoyeraal-Hreidarsson syndrome
Organism
Homo sapiens (human) [GN:hsa]
Gene
1460  
CSNK2B; casein kinase 2 beta [KO:K03115]
1457  
CSNK2A1; casein kinase 2 alpha 1 [KO:K03097] [EC:2.7.11.1]
1459  
CSNK2A2; casein kinase 2 alpha 2 [KO:K03097] [EC:2.7.11.1]
283106  
CSNK2A3; casein kinase 2 alpha 3 [KO:K03097] [EC:2.7.11.1]
65083  
NOL6; nucleolar protein 6 [KO:K14544]
27341  
RRP7A; ribosomal RNA processing 7 homolog A [KO:K14545]
23160  
WDR43; WD repeat domain 43 [KO:K14546]
84916  
UTP4; UTP4, small subunit processome component [KO:K14548]
84135  
UTP15; UTP15, small subunit processome component [KO:K14549]
55127  
HEATR1; HEAT repeat containing 1 [KO:K14550]
84128  
WDR75; WD repeat domain 75 [KO:K14552]
51096  
UTP18; UTP18, small subunit processome component [KO:K14553]
134430  
WDR36; WD repeat domain 36 [KO:K14554]
10607  
TBL3; transducin (beta)-like 3 [KO:K14555]
10885  
WDR3; WD repeat domain 3 [KO:K14556]
55813  
UTP6; UTP6, small subunit processome component [KO:K14557]
5822  
PWP2; PWP2 periodic tryptophan protein homolog (yeast) [KO:K14558]
10199  
MPHOSPH10; M-phase phosphoprotein 10 [KO:K14559]
643802  
u3 small nucleolar ribonucleoprotein protein MPP10-like; K14559 U3 small nucleolar RNA-associated protein MPP10 [KO:K14559]
55272  
IMP3; IMP3, U3 small nucleolar ribonucleoprotein [KO:K14560]
92856  
IMP4; IMP4 homolog, U3 small nucleolar ribonucleoprotein [KO:K14561]
6949  
TCOF1; treacle ribosome biogenesis factor 1 [KO:K14562]
2091  
FBL; fibrillarin [KO:K14563] [EC:2.1.1.-]
10528  
NOP56; NOP56 ribonucleoprotein [KO:K14564]
51602  
NOP58; NOP58 ribonucleoprotein [KO:K14565]
4809  
SNU13; SNU13 homolog, small nuclear ribonucleoprotein (U4/U6.U5) [KO:K12845]
1736  
DKC1; dyskerin pseudouridine synthase 1 [KO:K11131] [EC:5.4.99.-]
55651  
NHP2; NHP2 ribonucleoprotein [KO:K11129]
54433  
GAR1; GAR1 ribonucleoprotein [KO:K11128]
55505  
NOP10; NOP10 ribonucleoprotein [KO:K11130]
51077  
FCF1; FCF1 rRNA-processing protein [KO:K14566]
9724  
UTP14C; UTP14, small subunit processome component homolog C (S. cerevisiae) [KO:K14567]
10813  
UTP14A; UTP14A small subunit processome component [KO:K14567]
29102  
DROSHA; drosha ribonuclease III [KO:K03685] [EC:3.1.26.3]
10436  
EMG1; EMG1 N1-specific pseudouridine methyltransferase [KO:K14568] [EC:2.1.1.260]
9790  
BMS1; BMS1, ribosome biogenesis factor [KO:K14569]
55226  
NAT10; N-acetyltransferase 10 [KO:K14521] [EC:2.3.1.-]
10171  
RCL1; RNA terminal phosphate cyclase-like 1 [KO:K11108]
10940  
POP1; POP1 homolog, ribonuclease P/MRP subunit [KO:K01164] [EC:3.1.26.5]
10557  
RPP38; ribonuclease P/MRP 38kDa subunit [KO:K14523] [EC:3.1.26.5]
10775  
POP4; POP4 homolog, ribonuclease P/MRP subunit [KO:K03538] [EC:3.1.26.5]
51367  
POP5; POP5 homolog, ribonuclease P/MRP subunit [KO:K03537] [EC:3.1.26.5]
138716  
RPP25L; ribonuclease P/MRP 25kDa subunit-like [KO:K14525] [EC:3.1.26.5]
54913  
RPP25; ribonuclease P/MRP 25kDa subunit [KO:K14525] [EC:3.1.26.5]
10248  
POP7; POP7 homolog, ribonuclease P/MRP subunit [KO:K14527] [EC:3.1.26.5]
10556  
RPP30; ribonuclease P/MRP 30kDa subunit [KO:K03539] [EC:3.1.26.5]
10799  
RPP40; ribonuclease P/MRP 40kDa subunit [KO:K14530] [EC:3.1.26.5]
putative exonuclease GOR; K14570 RNA exonuclease 1 [EC:3.1.-.-] [KO:K14570] [EC:3.1.-.-]
57455  
REXO1; REX1, RNA exonuclease 1 homolog [KO:K14570] [EC:3.1.-.-]
putative exonuclease GOR; K14570 RNA exonuclease 1 [EC:3.1.-.-] [KO:K14570] [EC:3.1.-.-]
putative exonuclease GOR-like protein; K14570 RNA exonuclease 1 [EC:3.1.-.-] [KO:K14570] [EC:3.1.-.-]
81691  
exonuclease NEF-sp; K14570 RNA exonuclease 1 [EC:3.1.-.-] [KO:K14570] [EC:3.1.-.-]
putative exonuclease GOR; K14570 RNA exonuclease 1 [EC:3.1.-.-] [KO:K14570] [EC:3.1.-.-]
25996  
REXO2; RNA exonuclease 2 [KO:K13288] [EC:3.1.-.-]
54464  
XRN1; 5'-3' exoribonuclease 1 [KO:K12618] [EC:3.1.13.-]
22803  
XRN2; 5'-3' exoribonuclease 2 [KO:K12619] [EC:3.1.13.-]
23560  
GTPBP4; GTP binding protein 4 [KO:K06943]
29889  
GNL2; G protein nucleolar 2 [KO:K14537]
26354  
GNL3; G protein nucleolar 3 [KO:K14538]
54552  
GNL3L; G protein nucleolar 3 like [KO:K14538]
4931  
NVL; nuclear VCP-like [KO:K14571]
23195  
MDN1; midasin AAA ATPase 1 [KO:K14572]
55131  
RBM28; RNA binding motif protein 28 [KO:K14573]
28987  
NOB1; NIN1/PSMD8 binding protein 1 homolog [KO:K11883]
5901  
RAN; RAN, member RAS oncogene family [KO:K07936]
7514  
XPO1; exportin 1 [KO:K14290]
51068  
NMD3; NMD3 ribosome export adaptor [KO:K07562]
10482  
NXF1; nuclear RNA export factor 1 [KO:K14284]
56001  
NXF2; nuclear RNA export factor 2 [KO:K14284]
728343  
NXF2B; nuclear RNA export factor 2B [KO:K14284]
55998  
NXF5; nuclear RNA export factor 5 [KO:K14284]
56000  
NXF3; nuclear RNA export factor 3 [KO:K14284]
29107  
NXT1; nuclear transport factor 2-like export factor 1 [KO:K14285]
55916  
NXT2; nuclear transport factor 2-like export factor 2 [KO:K14285]
3692  
EIF6; eukaryotic translation initiation factor 6 [KO:K03264]
51119  
SBDS; SBDS ribosome assembly guanine nucleotide exchange factor [KO:K14574]
79631  
EFL1; elongation factor like GTPase 1 [KO:K14536] [EC:3.6.5.-]
55341  
LSG1; large 60S subunit nuclear export GTPase 1 [KO:K14539] [EC:3.6.1.-]
166378  
SPATA5; spermatogenesis associated 5 [KO:K14575]
AK6; adenylate kinase 6 [KO:K18532] [EC:2.7.4.3]
83732  
RIOK1; RIO kinase 1 [KO:K07178] [EC:2.7.11.1]
55781  
RIOK2; RIO kinase 2 [KO:K07179] [EC:2.7.11.1]
4549  
RNR1; s-rRNA [KO:K01979]
4550  
RNR2; l-rRNA [KO:K01982]
780851  
SNORD3A; small nucleolar RNA, C/D box 3A [KO:K14483]
780853  
SNORD3C; small nucleolar RNA, C/D box 3C [KO:K14483]
780852  
SNORD3B-2; small nucleolar RNA, C/D box 3B-2 [KO:K14483]
26851  
SNORD3B-1; small nucleolar RNA, C/D box 3B-1 [KO:K14483]
6023  
RMRP; RNA component of mitochondrial RNA processing endoribonuclease [KO:K14576]
Reference
  Authors
Freed EF, Bleichert F, Dutca LM, Baserga SJ
  Title
When ribosomes go bad: diseases of ribosome biogenesis.
  Journal
Mol Biosyst 6:481-93 (2010)
Reference
  Authors
Perez-Fernandez J, Roman A, De Las Rivas J, Bustelo XR, Dosil M
  Title
The 90S preribosome is a multimodular structure that is assembled through a hierarchical mechanism.
  Journal
Mol Cell Biol 27:5414-29 (2007)
Reference
  Authors
Strunk BS, Karbstein K
  Title
Powering through ribosome assembly.
  Journal
RNA 15:2083-104 (2009)
Reference
  Authors
Granneman S, Baserga SJ
  Title
Ribosome biogenesis: of knobs and RNA processing.
  Journal
Exp Cell Res 296:43-50 (2004)
Reference
  Authors
Kressler D, Hurt E, Bassler J
  Title
Driving ribosome assembly.
  Journal
Biochim Biophys Acta 1803:673-83 (2010)
KO pathway
 

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