KEGG   PATHWAY: caty04141
Entry
caty04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Cercocebus atys (sooty mangabey)
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
caty04141  Protein processing in endoplasmic reticulum
caty04141

Other DBs
GO: 0030433 0034976
Organism
Cercocebus atys (sooty mangabey) [GN:caty]
Gene
105571375  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
105571415  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
105571421  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
105571478  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
105571487  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
105571592  UBQLN2; ubiquilin-2 [KO:K04523]
105571621  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
105572435  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
105572491  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
105572503  TXNDC5; LOW QUALITY PROTEIN: thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
105572541  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X1 [KO:K03868] [EC:2.3.2.32]
105572871  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
105573177  ERP29; endoplasmic reticulum resident protein 29 isoform X1 [KO:K09586]
105573264  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
105573294  HSP90B1; endoplasmin [KO:K09487]
105573430  [KO:K03283]
105573431  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
105573482  [KO:K09586]
105573937  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
105574095  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
105574100  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
105574288  STUB1; E3 ubiquitin-protein ligase CHIP isoform X1 [KO:K09561] [EC:2.3.2.27]
105574474  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
105574475  DNAJC3; dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
105574630  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
105574708  ATXN3L; putative ataxin-3-like protein [KO:K11863] [EC:3.4.22.-]
105574898  ERO1LB; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
105575267  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
105575496  CRYAA; alpha-crystallin A chain [KO:K09541]
105575539  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
105575685  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
105575744  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
105576280  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
105576379  ERLEC1; LOW QUALITY PROTEIN: endoplasmic reticulum lectin 1 [KO:K14008]
105576775  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
105576799  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
105576805  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
105576962  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
105577453  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
105577545  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
105577552  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
105577576  TMEM258; transmembrane protein 258 [KO:K26502]
105577619  CAPN1; LOW QUALITY PROTEIN: calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
105578353  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
105578477  PREB; prolactin regulatory element-binding protein [KO:K14003]
105578556  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
105578632  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
105578645  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
105578883  UBQLN3; ubiquilin-3 [KO:K04523]
105579129  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
105579862  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
105579903  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
105580058  [KO:K03283]
105580097  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
105580591  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
105580607  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741]
105580862  FBXO2; F-box only protein 2 isoform X1 [KO:K10099]
105580865  FBXO6; F-box only protein 6 isoform X1 [KO:K10100]
105580960  UBE4B; ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
105581450  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA isoform X1 [KO:K01230] [EC:3.2.1.113]
105581530  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
105581722  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
105581837  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
105582026  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
105582046  RRBP1; LOW QUALITY PROTEIN: ribosome-binding protein 1 [KO:K14000]
105582064  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
105582185  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
105582310  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
105582355  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
105582495  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
105582602  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A isoform X1 [KO:K07151] [EC:2.4.99.18]
105582685  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
105582749  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
105582856  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
105583074  DERL2; derlin-2 isoform X1 [KO:K13989]
105583688  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
105583798  DNAJC1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
105584230  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
105584691  CUL1; cullin-1 isoform X1 [KO:K03347]
105584694  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
105585214  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
105585254  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
105585422  WFS1; LOW QUALITY PROTEIN: wolframin [KO:K14020]
105585732  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
105585780  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
105585866  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
105585875  SEC61A1; protein transport protein Sec61 subunit alpha isoform 1 [KO:K10956]
105585955  MARCH6; E3 ubiquitin-protein ligase MARCH6 isoform X1 [KO:K10661] [EC:2.3.2.27]
105586050  SEC62; translocation protein SEC62 [KO:K12275]
105586623  ERO1L; LOW QUALITY PROTEIN: ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
105587025  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
105587241  OS9; protein OS-9 isoform X1 [KO:K10088]
105587252  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
105587426  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
105587905  HSPH1; LOW QUALITY PROTEIN: heat shock protein 105 kDa [KO:K09485]
105588142  PDIA3; LOW QUALITY PROTEIN: protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
105588262  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
105588271  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
105588592  P4HB; protein disulfide-isomerase isoform X1 [KO:K09580] [EC:5.3.4.1]
105588642  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
105588695  CRYAB; alpha-crystallin B chain [KO:K09542]
105588760  [KO:K04741] [EC:3.4.22.-]
105588982  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
105589034  SEC63; translocation protein SEC63 homolog [KO:K09540]
105589095  SEC23A; protein transport protein Sec23A [KO:K14006]
105589128  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
105589221  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
105589230  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
105589238  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
105589581  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
105589854  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
105589982  LMAN1; protein ERGIC-53 isoform X1 [KO:K10080]
105590006  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
105590079  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
105590085  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
105590351  UBXN4; UBX domain-containing protein 4 [KO:K24348]
105590405  CANX; calnexin isoform X1 [KO:K08054]
105590417  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
105590750  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
105591024  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
105591301  PLAA; phospholipase A-2-activating protein [KO:K14018]
105591436  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
105591811  UBXN6; UBX domain-containing protein 6 [KO:K14011]
105592121  UGGT1; LOW QUALITY PROTEIN: UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
105592298  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
105593037  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
105593108  SVIP; small VCP/p97-interacting protein [KO:K14014]
105593255  BAK1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
105593523  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
105593978  VIMP; selenoprotein S [KO:K14025]
105594235  PARK2; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
105594499  SSR2; translocon-associated protein subunit beta isoform X1 [KO:K13250]
105594500  UBQLN4; ubiquilin-4 [KO:K04523]
105594961  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
105595039  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit isoform X1 [KO:K12670]
105595261  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
105595265  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
105595568  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
105596143  MAGT1; magnesium transporter protein 1 [KO:K19478]
105596524  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
105596582  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
105596717  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
105596862  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
105596920  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
105597026  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
105597272  SAR1A; GTP-binding protein SAR1a isoform X1 [KO:K07953] [EC:3.6.5.-]
105597305  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
105597336  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
105597391  SEC61A2; protein transport protein Sec61 subunit alpha isoform 2 [KO:K10956]
105597938  SSR3; translocon-associated protein subunit gamma [KO:K13251]
105598108  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
105598661  DERL1; derlin-1 isoform X1 [KO:K11519]
105598728  SSR4; translocon-associated protein subunit delta [KO:K04571]
105598733  BCAP31; B-cell receptor-associated protein 31 isoform X1 [KO:K14009]
105598800  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
105598836  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
105599385  DERL3; derlin-3 isoform X1 [KO:K13989]
105599611  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
105599767  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
105599779  SEC24A; LOW QUALITY PROTEIN: protein transport protein Sec24A [KO:K14007]
105599781  SAR1B; GTP-binding protein SAR1b isoform X1 [KO:K07953] [EC:3.6.5.-]
105599851  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
105599865  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
105600059  LMAN1L; protein ERGIC-53-like isoform X1 [KO:K10081]
105600400  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
105601292  UFD1L; ubiquitin fusion degradation protein 1 homolog isoform X1 [KO:K14016]
105601485  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
105601547  CALR; calreticulin [KO:K08057]
105601548  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
105601590  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
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
caty00510  N-Glycan biosynthesis
caty03050  Proteasome
caty03060  Protein export
caty04210  Apoptosis
caty04930  Type II diabetes mellitus
caty05010  Alzheimer disease
caty05012  Parkinson disease
caty05020  Prion disease
KO pathway
ko04141   

DBGET integrated database retrieval system