KEGG   PATHWAY: csab04141
Entry
csab04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Chlorocebus sabaeus (green monkey)
Description
The endoplasmic reticulum (ER) is a subcellular organelle where proteins are folded with the help of lumenal chaperones. Newly synthesized peptides enter the ER via the sec61 pore and are glycosylated. Correctly folded proteins are packaged into transport vesicles that shuttle them to the Golgi complex. Misfolded proteins are retained within the ER lumen in complex with molecular chaperones. Proteins that are terminally misfolded bind to BiP and are directed toward degradation through the proteasome in a process called ER-associated degradation (ERAD). Accumulation of misfolded proteins in the ER causes ER stress and activates a signaling pathway called the unfolded protein response (UPR). In certain severe situations, however, the protective mechanisms activated by the UPR are not sufficient to restore normal ER function and cells die by apoptosis.
Class
Genetic Information Processing; Folding, sorting and degradation
Pathway map
csab04141  Protein processing in endoplasmic reticulum
csab04141

Other DBs
GO: 0030433 0034976
Organism
Chlorocebus sabaeus (green monkey) [GN:csab]
Gene
103214241  HSPH1; heat shock protein family H (Hsp110) member 1 [KO:K09485]
103214470  SEL1L2; SEL1L2 ERAD E3 ligase adaptor subunit [KO:K14026]
103214677  DNAJC3; DnaJ heat shock protein family (Hsp40) member C3 [KO:K09523]
103214678  UGGT2; UDP-glucose glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
103214724  RRBP1; ribosome binding protein 1 [KO:K14000]
103214961  SEC23B; Sec23 homolog B, coat complex II component [KO:K14006]
103215005  MARCH6; membrane associated ring-CH-type finger 6 [KO:K10661] [EC:2.3.2.27]
103215375  TUSC3; tumor suppressor candidate 3 [KO:K19478]
103215585  UBXN8; UBX domain protein 8 [KO:K24351]
103215842  NSFL1C; NSFL1 cofactor [KO:K14012]
103215844  MAPK8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
103215992  SEC24C; SEC24 homolog C, COPII coat complex component [KO:K14007]
103216025  DNAJB12; DnaJ heat shock protein family (Hsp40) member B12 [KO:K09518]
103216060  SAR1A; secretion associated Ras related GTPase 1A [KO:K07953] [EC:3.6.5.-]
103216166  UBE2D1; ubiquitin conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
103216389  SEC31B; SEC31 homolog B, COPII coat complex component [KO:K14005]
103216865  UGGT1; UDP-glucose glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
103216988  UBXN4; UBX domain protein 4 [KO:K24348]
103217393  NFE2L2; nuclear factor, erythroid 2 like 2 [KO:K05638]
103217465  DNAJC10; DnaJ heat shock protein family (Hsp40) member C10 [KO:K09530] [EC:1.8.4.-]
103217906  DNAJB2; DnaJ heat shock protein family (Hsp40) member B2 [KO:K09508]
103219057  RAD23B; RAD23 homolog B, nucleotide excision repair protein [KO:K10839]
103219137  SEC61B; Sec61 translocon beta subunit [KO:K09481]
103219235  VCP; valosin containing protein [KO:K13525]
103219289  BAG1; BCL2 associated athanogene 1 [KO:K09555]
103219299  DNAJA1; DnaJ heat shock protein family (Hsp40) member A1 [KO:K09502]
103219332  PLAA; phospholipase A2 activating protein [KO:K14018]
103219534  [KO:K09541]
103219664  UBQLN1; ubiquilin 1 [KO:K04523]
103219984  UBE2G2; ubiquitin conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
103220027  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
103220073  [KO:K04571]
103220270  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
103220604  DNAJC5G; DnaJ heat shock protein family (Hsp40) member C5 gamma [KO:K09525]
103220613  PREB; prolactin regulatory element binding [KO:K14003]
103220679  UBXN2A; UBX domain protein 2A [KO:K24349]
103220818  PDIA6; protein disulfide isomerase family A member 6 [KO:K09584] [EC:5.3.4.1]
103221093  DNAJB11; DnaJ heat shock protein family (Hsp40) member B11 [KO:K09517]
103221273  SEC62; SEC62 homolog, preprotein translocation factor [KO:K12275]
103221436  HSP90AB1; heat shock protein 90 alpha family class B member 1 [KO:K04079]
103221651  BAK1; BCL2 antagonist/killer 1 [KO:K14021]
103221708  RNF5; ring finger protein 5 [KO:K10666] [EC:2.3.2.27]
103221718  ATF6B; activating transcription factor 6 beta [KO:K09049]
103221743  HSPA1B; heat shock protein family A (Hsp70) member 1B [KO:K03283]
103221744  HSPA1L; heat shock protein family A (Hsp70) member 1 like [KO:K03283]
103221745  HSPA1A; heat shock protein family A (Hsp70) member 1A [KO:K03283]
103222206  TXNDC5; thioredoxin domain containing 5 [KO:K13984] [EC:5.3.4.1]
103222217  SSR1; signal sequence receptor subunit 1 [KO:K13249]
103223000  UFD1L; ubiquitin fusion degradation 1 like (yeast) [KO:K14016]
103223026  DERL3; derlin 3 [KO:K13989]
103223125  XBP1; X-box binding protein 1 [KO:K09027]
103223183  RNF185; ring finger protein 185 [KO:K10666] [EC:2.3.2.27]
103223345  ATF4; activating transcription factor 4 [KO:K04374]
103223367  RBX1; ring-box 1 [KO:K03868] [EC:2.3.2.32]
103223664  ATF6; activating transcription factor 6 [KO:K09054]
103223677  HSPA6; heat shock protein family A (Hsp70) member 6 [KO:K03283]
103223863  SSR2; signal sequence receptor subunit 2 [KO:K13250]
103223891  KRTCAP2; keratinocyte associated protein 2 [KO:K28202]
103224125  MAN1A2; mannosidase alpha class 1A member 2 [KO:K01230] [EC:3.2.1.113]
103225120  [KO:K19478]
103225352  MAN1C1; mannosidase alpha class 1C member 1 [KO:K01230] [EC:3.2.1.113]
103225449  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase non-catalytic subunit [KO:K12670]
103225604  FBXO6; F-box protein 6 [KO:K10100]
103225605  FBXO2; F-box protein 2 [KO:K10099]
103225621  STUB1; STIP1 homology and U-box containing protein 1 [KO:K09561] [EC:2.3.2.27]
103225813  UBE2J2; ubiquitin conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
103225869  UBQLN4; ubiquilin 4 [KO:K04523]
103225984  UBE4B; ubiquitination factor E4B [KO:K10597] [EC:2.3.2.27]
103226057  SEC61G; Sec61 translocon gamma subunit [KO:K07342]
103226151  UBE2D4; ubiquitin conjugating enzyme E2 D4 (putative) [KO:K06689] [EC:2.3.2.23]
103227180  CUL1; cullin 1 [KO:K03347]
103227184  PDIA4; protein disulfide isomerase family A member 4 [KO:K09582] [EC:5.3.4.1]
103227949  EDEM1; ER degradation enhancing alpha-mannosidase like protein 1 [KO:K10084]
103228003  SEC13; SEC13 homolog, nuclear pore and COPII coat complex component [KO:K14004]
103228090  RPN1; ribophorin I [KO:K12666]
103228100  SEC61A1; Sec61 translocon alpha 1 subunit [KO:K10956]
103228869  SEC23A; Sec23 homolog A, coat complex II component [KO:K14006]
103228991  ERO1A; endoplasmic reticulum oxidoreductase 1 alpha [KO:K10950] [EC:1.8.4.-]
103229205  EIF2S1; eukaryotic translation initiation factor 2 subunit alpha [KO:K03237]
103229454  SEL1L; SEL1L ERAD E3 ligase adaptor subunit [KO:K14026]
103229563  ATXN3; ataxin 3 [KO:K11863] [EC:3.4.22.-]
103229726  HSP90AA1; heat shock protein 90 alpha family class A member 1 [KO:K04079]
103230023  CAPN2; calpain 2 [KO:K03853] [EC:3.4.22.53]
103230099  SYVN1; synoviolin 1 [KO:K10601] [EC:2.3.2.27]
103230180  YOD1; YOD1 deubiquitinase [KO:K13719] [EC:3.1.2.-]
103230332  CAPN1; calpain 1 [KO:K01367] [EC:3.4.22.52]
103230438  EDEM3; ER degradation enhancing alpha-mannosidase like protein 3 [KO:K10086]
103230810  ERO1B; endoplasmic reticulum oxidoreductase 1 beta [KO:K10976] [EC:1.8.4.-]
103230925  [KO:K03283]
103231289  SELENOS; selenoprotein S [KO:K14025]
103231439  DAD1; defender against cell death 1 [KO:K12668]
103231613  ATXN3L; ataxin 3 like [KO:K11863] [EC:3.4.22.-]
103231700  MBTPS2; membrane bound transcription factor peptidase, site 2 [KO:K07765] [EC:3.4.24.85]
103232056  UBQLN2; ubiquilin 2 [KO:K04523]
103232232  [KO:K19478]
103232247  DNAJA2; DnaJ heat shock protein family (Hsp40) member A2 [KO:K09503]
103232800  SSR4; signal sequence receptor subunit 4 [KO:K04571]
103232989  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
103233021  AMFR; autocrine motility factor receptor [KO:K10636] [EC:2.3.2.36]
103233048  HERPUD1; homocysteine inducible ER protein with ubiquitin like domain 1 [KO:K14027]
103233385  MBTPS1; membrane bound transcription factor peptidase, site 1 [KO:K08653] [EC:3.4.21.112]
103233702  UBXN6; UBX domain protein 6 [KO:K14011]
103233823  MAP2K7; mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
103233930  PRKCSH; protein kinase C substrate 80K-H [KO:K08288]
103233953  UBXN1; UBX domain protein 1 [KO:K24348]
103233997  GANAB; glucosidase II alpha subunit [KO:K05546] [EC:3.2.1.207]
103234003  CALR; calreticulin [KO:K08057]
103234004  RAD23A; RAD23 homolog A, nucleotide excision repair protein [KO:K10839]
103234043  DNAJB1; DnaJ heat shock protein family (Hsp40) member B1 [KO:K09507]
103234205  TMEM258; transmembrane protein 258 [KO:K26502]
103235004  BAX; BCL2 associated X, apoptosis regulator [KO:K02159]
103235298  HSPBP1; HSPA (Hsp70) binding protein 1 [KO:K09562]
103235893  SEC31A; SEC31 homolog A, COPII coat complex component [KO:K14005]
103235918  MAPK10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
103236056  UBE2D3; ubiquitin conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
103236093  OSTC; oligosaccharyltransferase complex non-catalytic subunit [KO:K26501]
103236101  SEC24B; SEC24 homolog B, COPII coat complex component [KO:K14007]
103236163  TRAM1L1; translocation associated membrane protein 1-like 1 [KO:K14010]
103236172  SEC24D; SEC24 homolog D, COPII coat complex component [KO:K14007]
103236239  HSPA4L; heat shock protein family A (Hsp70) member 4 like [KO:K09485]
103236665  [KO:K04079]
103236897  DNAJC5B; DnaJ heat shock protein family (Hsp40) member C5 beta [KO:K09525]
103236949  TRAM1; translocation associated membrane protein 1 [KO:K14010]
103237376  DERL1; derlin 1 [KO:K11519]
103237891  [KO:K10956]
103237989  DNAJC1; DnaJ heat shock protein family (Hsp40) member C1 [KO:K09521]
103238566  DDIT3; DNA damage inducible transcript 3 [KO:K04452]
103238586  OS9; OS9, endoplasmic reticulum lectin [KO:K10088]
103238741  SVIP; small VCP interacting protein [KO:K14014]
103238976  HSP90B1; heat shock protein 90 beta family member 1 [KO:K09487]
103239025  CKAP4; cytoskeleton associated protein 4 [KO:K13999]
103239138  ERP29; endoplasmic reticulum protein 29 [KO:K09586]
103239582  MAN1B1; mannosidase alpha class 1B member 1 [KO:K23741]
103239600  TRAF2; TNF receptor associated factor 2 [KO:K03173] [EC:2.3.2.27]
103239874  HSPA5; heat shock protein family A (Hsp70) member 5 [KO:K09490] [EC:3.6.4.10]
103240101  UBE2J1; ubiquitin conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
103240310  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
103240478  MAN1A1; mannosidase alpha class 1A member 1 [KO:K01230] [EC:3.2.1.113]
103240862  PARK2; parkin RBR E3 ubiquitin protein ligase [KO:K04556] [EC:2.3.2.31]
103241021  SEC63; SEC63 homolog, protein translocation regulator [KO:K09540]
103241350  EIF2AK3; eukaryotic translation initiation factor 2 alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
103241428  SSR3; signal sequence receptor subunit 3 [KO:K13251]
103241778  NGLY1; N-glycanase 1 [KO:K01456] [EC:3.5.1.52]
103241813  STT3B; STT3B, catalytic subunit of the oligosaccharyltransferase complex [KO:K07151] [EC:2.4.99.18]
103242182  UBE2G1; ubiquitin conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
103242236  DERL2; derlin 2 [KO:K13989]
103242453  DNAJC5; DnaJ heat shock protein family (Hsp40) member C5 [KO:K09525]
103243204  ERN1; endoplasmic reticulum to nucleus signaling 1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
103243723  NPLOC4; NPL4 homolog, ubiquitin recognition factor [KO:K14015]
103243733  P4HB; prolyl 4-hydroxylase subunit beta [KO:K09580] [EC:5.3.4.1]
103243973  BAG2; BCL2 associated athanogene 2 [KO:K09556]
103244166  BCL2; BCL2, apoptosis regulator [KO:K02161]
103244199  LMAN1; lectin, mannose binding 1 [KO:K10080]
103244544  SKP1; S-phase kinase associated protein 1 [KO:K03094]
103244554  SAR1B; secretion associated Ras related GTPase 1B [KO:K07953] [EC:3.6.5.-]
103244556  [KO:K14007]
103244629  SIL1; SIL1 nucleotide exchange factor [KO:K14001]
103244643  UBE2D2; ubiquitin conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
103245049  LMAN2; lectin, mannose binding 2 [KO:K10082]
103245105  CANX; calnexin [KO:K08054]
103245120  MAPK9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
103245326  LMAN1L; lectin, mannose binding 1 like [KO:K10081]
103245734  PDIA3; protein disulfide isomerase family A member 3 [KO:K08056] [EC:5.3.4.1]
103246235  [KO:K06689] [EC:2.3.2.23]
103246405  WFS1; wolframin ER transmembrane glycoprotein [KO:K14020]
103246432  RPN2; ribophorin II [KO:K12667]
103247895  UBQLN3; ubiquilin 3 [KO:K04523]
103248368  [KO:K04741] [EC:3.4.22.-]
103248454  CRYAB; crystallin alpha B [KO:K09542]
103248616  HYOU1; hypoxia up-regulated 1 [KO:K09486]
103248693  HSPA8; heat shock protein family A (Hsp70) member 8 [KO:K03283]
103248767  STT3A; STT3A, catalytic subunit of the oligosaccharyltransferase complex [KO:K07151] [EC:2.4.99.18]
103248949  [KO:K10085]
Compound
C00076  Calcium cation
G00009  
G00010  
G00011  
G00012  
G10694  
Reference
PMID:19491040
  Authors
Naidoo N
  Title
ER and aging-Protein folding and the ER stress response.
  Journal
Ageing Res Rev 8:150-9 (2009)
DOI:10.1016/j.arr.2009.03.001
Reference
PMID:18023214
  Authors
Malhotra JD, Kaufman RJ
  Title
The endoplasmic reticulum and the unfolded protein response.
  Journal
Semin Cell Dev Biol 18:716-31 (2007)
DOI:10.1016/j.semcdb.2007.09.003
Reference
PMID:20347046
  Authors
Maattanen P, Gehring K, Bergeron JJ, Thomas DY
  Title
Protein quality control in the ER: the recognition of misfolded proteins.
  Journal
Semin Cell Dev Biol 21:500-11 (2010)
DOI:10.1016/j.semcdb.2010.03.006
Reference
PMID:20219571
  Authors
Stolz A, Wolf DH
  Title
Endoplasmic reticulum associated protein degradation: a chaperone assisted journey to hell.
  Journal
Biochim Biophys Acta 1803:694-705 (2010)
DOI:10.1016/j.bbamcr.2010.02.005
Reference
PMID:20112977
  Authors
Dejgaard K, Theberge JF, Heath-Engel H, Chevet E, Tremblay ML, Thomas DY
  Title
Organization of the Sec61 translocon, studied by high resolution native electrophoresis.
  Journal
J Proteome Res 9:1763-71 (2010)
DOI:10.1021/pr900900x
Related
pathway
csab00510  N-Glycan biosynthesis
csab03050  Proteasome
csab03060  Protein export
csab04210  Apoptosis
csab04930  Type II diabetes mellitus
csab05010  Alzheimer disease
csab05012  Parkinson disease
csab05020  Prion disease
KO pathway
ko04141   

DBGET integrated database retrieval system