KEGG   PATHWAY: ko03008
Entry
ko03008                     Pathway                                

Name
Ribosome biogenesis in eukaryotes
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
Pathway map
ko03008  Ribosome biogenesis in eukaryotes
ko03008

Disease
H00063  Spinocerebellar ataxia (SCA)
H00439  Shwachman-Diamond syndrome
H00507  Dyskeratosis congenita
H00518  Metaphyseal dysplasia without hypotrichosis
H00610  Treacher Collins syndrome
H00612  Primary open angle glaucoma
H00616  Bowen-Conradi syndrome
H00621  Alopecia neurologic defects and endocrinopathy syndrome
H00788  Hoyeraal-Hreidarsson syndrome
H01896  Aplasia cutis congenita
H01966  Cartilage-hair hypoplasia
H01967  Anauxetic dysplasia
H02194  North American Indian childhood cirrhosis
Orthology
K03115  casein kinase II subunit beta
K03097  casein kinase II subunit alpha [EC:2.7.11.1]
K14544  U3 small nucleolar RNA-associated protein 22
K14545  ribosomal RNA-processing protein 7
K14546  U3 small nucleolar RNA-associated protein 5
K14547  U3 small nucleolar RNA-associated protein 8
K14548  U3 small nucleolar RNA-associated protein 4
K14549  U3 small nucleolar RNA-associated protein 15
K14550  U3 small nucleolar RNA-associated protein 10
K14551  U3 small nucleolar RNA-associated protein 9
K14552  NET1-associated nuclear protein 1 (U3 small nucleolar RNA-associated protein 17)
K14553  U3 small nucleolar RNA-associated protein 18
K14554  U3 small nucleolar RNA-associated protein 21
K14555  U3 small nucleolar RNA-associated protein 13
K14556  U3 small nucleolar RNA-associated protein 12
K14557  U3 small nucleolar RNA-associated protein 6
K14558  periodic tryptophan protein 2
K14559  U3 small nucleolar RNA-associated protein MPP10
K14560  U3 small nucleolar ribonucleoprotein protein IMP3
K14561  U3 small nucleolar ribonucleoprotein protein IMP4
K14562  Treacher Collins syndrome protein
K14563  rRNA 2'-O-methyltransferase fibrillarin [EC:2.1.1.-]
K14564  nucleolar protein 56
K14565  nucleolar protein 58
K12845  U4/U6 small nuclear ribonucleoprotein SNU13
K11131  H/ACA ribonucleoprotein complex subunit 4 [EC:5.4.99.-]
K11129  H/ACA ribonucleoprotein complex subunit 2
K11128  H/ACA ribonucleoprotein complex subunit 1
K11130  H/ACA ribonucleoprotein complex subunit 3
K14566  U3 small nucleolar RNA-associated protein 24
K14567  U3 small nucleolar RNA-associated protein 14
K03685  ribonuclease III [EC:3.1.26.3]
K14568  rRNA small subunit pseudouridine methyltransferase Nep1 [EC:2.1.1.260]
K14569  ribosome biogenesis protein BMS1
K14521  N-acetyltransferase 10 [EC:2.3.1.-]
K11108  RNA 3'-terminal phosphate cyclase-like protein
K01164  ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
K14522  ribonuclease P/MRP protein subunit POP3 [EC:3.1.26.5]
K14523  ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
K03538  ribonuclease P protein subunit POP4 [EC:3.1.26.5]
K03537  ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
K14524  ribonuclease P/MRP protein subunit POP6 [EC:3.1.26.5]
K14525  ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
K14526  ribonuclease P/MRP protein subunit POP7 [EC:3.1.26.5]
K14527  ribonuclease P/MRP protein subunit RPP20 [EC:3.1.26.5]
K14528  ribonuclease P/MRP protein subunit POP8 [EC:3.1.26.5]
K03539  ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
K14531  ribonuclease MRP protein subunit SNM1
K14532  ribonuclease MRP protein subunit RMP1
K14530  ribonucleases P/MRP protein subunit RPP40 [EC:3.1.26.5]
K14570  RNA exonuclease 1 [EC:3.1.-.-]
K13288  oligoribonuclease [EC:3.1.-.-]
K12618  5'-3' exoribonuclease 1 [EC:3.1.13.-]
K12619  5'-3' exoribonuclease 2 [EC:3.1.13.-]
K06943  nucleolar GTP-binding protein
K14537  nuclear GTP-binding protein
K14538  nuclear GTP-binding protein
K14571  ribosome biogenesis ATPase
K14572  midasin
K14573  nucleolar protein 4
K11883  RNA-binding protein NOB1
K14758  casein kinase I homolog HRR25 [EC:2.7.11.1]
K07936  GTP-binding nuclear protein Ran
K14290  exportin-1
K07562  nonsense-mediated mRNA decay protein 3
K14284  nuclear RNA export factor
K14543  mRNA transport regulator MTR2
K14285  NTF2-related export protein 1/2
K03264  translation initiation factor 6
K14574  ribosome maturation protein SDO1
K14536  ribosome assembly protein 1 [EC:3.6.5.-]
K14539  large subunit GTPase 1 [EC:3.6.1.-]
K14575  AAA family ATPase
K18532  adenylate kinase [EC:2.7.4.3]
K07178  RIO kinase 1 [EC:2.7.11.1]
K07179  RIO kinase 2 [EC:2.7.11.1]
K01979  small subunit ribosomal RNA
K01986  large subunit ribosomal RNA, 5.8S
K01982  large subunit ribosomal RNA
K01981  large subunit ribosomal RNA, 5S
K14483  U3 small nucleolar RNA
K14576  small nucleolar RNA MRP
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)
DOI:10.1039/b919670f
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)
DOI:10.1128/MCB.00380-07
Reference
  Authors
Strunk BS, Karbstein K
  Title
Powering through ribosome assembly.
  Journal
RNA 15:2083-104 (2009)
DOI:10.1261/rna.1792109
Reference
  Authors
Granneman S, Baserga SJ
  Title
Ribosome biogenesis: of knobs and RNA processing.
  Journal
Exp Cell Res 296:43-50 (2004)
DOI:10.1016/j.yexcr.2004.03.016
Reference
  Authors
Kressler D, Hurt E, Bassler J
  Title
Driving ribosome assembly.
  Journal
Biochim Biophys Acta 1803:673-83 (2010)
DOI:10.1016/j.bbamcr.2009.10.009
Related
pathway
ko03010  Ribosome

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