KEGG   PATHWAY: cimi04141
Entry
cimi04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Cebus imitator (Panamanian white-faced capuchin)
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
cimi04141  Protein processing in endoplasmic reticulum
cimi04141

Other DBs
GO: 0030433 0034976
Organism
Cebus imitator (Panamanian white-faced capuchin) [GN:cimi]
Gene
108281403  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 [KO:K10661] [EC:2.3.2.27]
108281917  DNAJB1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
108281985  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
108282026  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
108282029  CALR; calreticulin [KO:K08057]
108282238  DERL3; derlin-3 isoform X1 [KO:K13989]
108282574  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
108282619  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
108283240  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
108283291  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
108283651  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
108283904  PPP1R15A; protein phosphatase 1 regulatory subunit 15A isoform X1 [KO:K14019]
108283911  BAX; apoptosis regulator BAX [KO:K02159]
108284042  [KO:K04079]
108284112  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
108284245  [KO:K14001]
108284368  [KO:K19478]
108284658  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
108284997  SEC62; translocation protein SEC62 [KO:K12275]
108285450  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
108285466  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
108285616  CUL1; cullin-1 [KO:K03347]
108285622  PDIA4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
108285677  UBE4B; ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
108286226  TMEM258; transmembrane protein 258 [KO:K26502]
108286286  UBXN1; UBX domain-containing protein 1 [KO:K24348]
108286292  GANAB; neutral alpha-glucosidase AB isoform X2 [KO:K05546] [EC:3.2.1.207]
108286341  FBXO2; F-box only protein 2 [KO:K10099]
108286382  FBXO6; F-box only protein 6 [KO:K10100]
108286678  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
108287107  [KO:K02159]
108287256  MAN1B1; LOW QUALITY PROTEIN: endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
108287258  TRAF2; TNF receptor-associated factor 2 isoform X2 [KO:K03173] [EC:2.3.2.27]
108287481  SEL1L; protein sel-1 homolog 1 [KO:K14026]
108287675  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
108287849  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
108288015  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
108288370  UBQLN3; ubiquilin-3 isoform X2 [KO:K04523]
108289197  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
108289249  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
108289311  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
108289382  ATXN3L; ataxin-3-like protein [KO:K11863] [EC:3.4.22.-]
108289668  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
108289804  SEC24B; protein transport protein Sec24B [KO:K14007]
108290216  SSR2; translocon-associated protein subunit beta isoform X1 [KO:K13250]
108290218  UBQLN4; ubiquilin-4 [KO:K04523]
108290508  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
108290619  [KO:K04079]
108290730  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
108290782  SEC24D; protein transport protein Sec24D [KO:K14007]
108290876  STUB1; E3 ubiquitin-protein ligase CHIP isoform X1 [KO:K09561] [EC:2.3.2.27]
108291482  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
108291501  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
108291538  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
108291589  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
108291637  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
108291730  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
108291804  [KO:K03283]
108291907  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
108291925  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
108292029  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
108292055  SEC23B; protein transport protein Sec23B [KO:K14006]
108292072  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
108292110  [KO:K03283]
108292158  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
108292479  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
108292582  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
108292745  [KO:K14014]
108293362  UBXN4; UBX domain-containing protein 4 [KO:K24348]
108293602  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
108293618  HSPA6; heat shock 70 kDa protein 6 [KO:K03283]
108293793  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
108293817  [KO:K04374]
108293913  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
108293970  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
108294539  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
108294750  BCAP31; B-cell receptor-associated protein 31 isoform X1 [KO:K14009]
108294759  SSR4; translocon-associated protein subunit delta [KO:K04571]
108294875  UBQLN2; ubiquilin-2 [KO:K04523]
108295209  CANX; calnexin [KO:K08054]
108295228  MAPK9; mitogen-activated protein kinase 9 isoform X2 [KO:K04440] [EC:2.7.11.24]
108295469  CRYAB; alpha-crystallin B chain isoform X1 [KO:K09542]
108295573  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
108295615  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
108296025  HSPH1; heat shock protein 105 kDa [KO:K09485]
108296428  SELENOS; selenoprotein S [KO:K14025]
108296945  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
108296967  LMAN1L; protein ERGIC-53-like isoform X1 [KO:K10081]
108297235  MAGT1; magnesium transporter protein 1 [KO:K19478]
108297517  PLAA; phospholipase A-2-activating protein [KO:K14018]
108297764  DERL2; derlin-2 isoform X1 [KO:K13989]
108299400  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
108299802  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
108299822  [KO:K03283]
108299823  [KO:K03283]
108299825  [KO:K03283]
108300037  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
108300111  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
108300227  SSR3; translocon-associated protein subunit gamma [KO:K13251]
108300593  [KO:K03094]
108300626  HYOU1; hypoxia up-regulated protein 1 isoform X2 [KO:K09486]
108300687  HSPA8; LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein [KO:K03283]
108300784  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A isoform X1 [KO:K07151] [EC:2.4.99.18]
108300927  [KO:K03094]
108301119  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
108301266  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
108301577  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
108301660  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
108301675  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
108302095  YOD1; ubiquitin thioesterase OTU1 isoform X1 [KO:K13719] [EC:3.1.2.-]
108302152  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
108302703  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
108302852  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
108302982  TXNDC5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
108302990  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
108303075  [KO:K09502]
108303877  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
108303918  [KO:K03283]
108304038  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
108304287  UBXN6; UBX domain-containing protein 6 [KO:K14011]
108304574  LMAN1; protein ERGIC-53 [KO:K10080]
108304605  BCL2; apoptosis regulator Bcl-2 isoform X2 [KO:K02161]
108305275  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
108305413  [KO:K11718] [EC:2.4.1.-]
108305507  SEC31A; protein transport protein Sec31A [KO:K14005]
108305646  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
108305664  [KO:K01228] [EC:3.2.1.106]
108305837  [KO:K03283]
108305954  DERL1; derlin-1 isoform X1 [KO:K11519]
108306089  SEC23A; protein transport protein Sec23A [KO:K14006]
108306364  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
108306996  [KO:K08056] [EC:5.3.4.1]
108306997  SEC24A; protein transport protein Sec24A [KO:K14007]
108307137  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
108307146  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
108307320  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
108307370  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
108308688  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
108309099  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
108309237  TUSC3; tumor suppressor candidate 3 [KO:K19478]
108309549  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
108309731  DDIT3; LOW QUALITY PROTEIN: DNA damage-inducible transcript 3 protein [KO:K04452]
108309740  OS9; protein OS-9 isoform X1 [KO:K10088]
108310018  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
108310057  [KO:K14001]
108310071  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
108310246  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
108310251  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
108310303  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
108311114  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
108311212  CRYAA; alpha-crystallin A chain [KO:K09541]
108311279  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
108311625  WFS1; wolframin [KO:K14020]
108312236  [KO:K14009]
108312418  HSP90B1; endoplasmin [KO:K09487]
108312668  [KO:K03094]
108312713  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
108312882  [KO:K26501]
108312889  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
108313560  [KO:K03094]
108313740  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
108313788  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
108313921  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
108314043  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
108314135  ERP29; endoplasmic reticulum resident protein 29 isoform X1 [KO:K09586]
108314196  [KO:K03283]
108314228  [KO:K13525]
108314614  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
108314636  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
108314713  SEC63; translocation protein SEC63 homolog [KO:K09540]
108314972  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
108315231  [KO:K12668]
108315232  [KO:K12668]
108315292  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
108315343  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
108315471  [KO:K28202]
108315696  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
108315721  [KO:K09502]
108315917  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
108315928  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
108316615  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
108316809  ATXN3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
108317063  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
108317187  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
108317683  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
108317729  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
108317780  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
108318075  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
108318107  SAR1A; LOW QUALITY PROTEIN: GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
108318239  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
119468660  [KO:K03237]
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
cimi00510  N-Glycan biosynthesis
cimi03050  Proteasome
cimi03060  Protein export
cimi04210  Apoptosis
cimi04930  Type II diabetes mellitus
cimi05010  Alzheimer disease
cimi05012  Parkinson disease
cimi05020  Prion disease
KO pathway
ko04141   

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