KEGG   PATHWAY: tfn04141
Entry
tfn04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Trachypithecus francoisi (Francois's langur)
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
tfn04141  Protein processing in endoplasmic reticulum
tfn04141

Other DBs
GO: 0030433 0034976
Organism
Trachypithecus francoisi (Francois's langur) [GN:tfn]
Gene
117062056  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
117062173  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
117062310  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
117062534  SSR4; translocon-associated protein subunit delta [KO:K04571]
117062567  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
117062574  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
117062641  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
117062726  PDIA6; protein disulfide-isomerase A6 isoform X1 [KO:K09584] [EC:5.3.4.1]
117063594  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
117063596  DNAJC3; dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
117064344  CANX; calnexin isoform X1 [KO:K08054]
117064749  MARCHF6; E3 ubiquitin-protein ligase MARCH6 isoform X1 [KO:K10661] [EC:2.3.2.27]
117065339  LMAN2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
117065429  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
117065774  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
117065789  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
117065851  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
117065854  SAR1B; GTP-binding protein SAR1b isoform X1 [KO:K07953] [EC:3.6.5.-]
117065865  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
117066825  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
117066838  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
117067424  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
117067841  DERL2; derlin-2 isoform X1 [KO:K13989]
117067894  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
117068394  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
117068405  SEC23B; protein transport protein Sec23B [KO:K14006]
117068422  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
117068439  SEL1L2; protein sel-1 homolog 2 isoform X1 [KO:K14026]
117068574  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
117068689  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
117068746  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
117068816  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
117069132  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
117069233  SEL1L; protein sel-1 homolog 1 [KO:K14026]
117069444  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
117069498  [KO:K03094]
117069641  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
117069698  [KO:K04079]
117069787  SEC23A; protein transport protein Sec23A [KO:K14006]
117070003  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
117070120  SELENOS; selenoprotein S isoform X1 [KO:K14025]
117070360  LMAN1L; protein ERGIC-53-like [KO:K10081]
117070762  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
117071315  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
117071348  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
117071526  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
117072040  PDIA4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
117073295  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
117073311  [KO:K09555]
117073499  CUL1; cullin-1 [KO:K03347]
117073503  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
117074239  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
117074354  UBXN6; UBX domain-containing protein 6 [KO:K14011]
117074457  [KO:K04079]
117074467  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
117074470  CALR; calreticulin [KO:K08057]
117074574  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
117075199  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
117075348  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
117075695  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
117076179  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
117076185  DNAJB1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
117076224  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
117076376  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
117076399  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
117076493  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
117076723  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
117076724  SSR2; translocon-associated protein subunit beta [KO:K13250]
117077247  MAGT1; LOW QUALITY PROTEIN: magnesium transporter protein 1 [KO:K19478]
117077316  [KO:K26502]
117077330  [KO:K07765] [EC:3.4.24.85]
117077399  UBQLN2; ubiquilin-2 [KO:K04523]
117078063  ATXN3L; LOW QUALITY PROTEIN: ataxin-3-like protein [KO:K11863] [EC:3.4.22.-]
117078185  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
117078944  DNAJC5B; dnaJ homolog subfamily C member 5B isoform X1 [KO:K09525]
117079439  DERL1; derlin-1 isoform X1 [KO:K11519]
117079486  TRAM1; translocating chain-associated membrane protein 1 isoform X1 [KO:K14010]
117079501  SSR3; translocon-associated protein subunit gamma [KO:K13251]
117080263  TMEM258; transmembrane protein 258 [KO:K26502]
117080378  SVIP; small VCP/p97-interacting protein [KO:K14014]
117080400  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
117080866  UBQLN3; ubiquilin-3 [KO:K04523]
117080886  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
117081091  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
117081099  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
117081200  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
117081565  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
117081573  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
117081984  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
117082169  [KO:K09502]
117082352  [KO:K26501]
117082490  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
117082577  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
117082641  SEC24C; LOW QUALITY PROTEIN: protein transport protein Sec24C [KO:K14007]
117082707  [KO:K04079]
117082742  DNAJC1; LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 1 [KO:K09521]
117082861  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
117082864  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
117083182  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
117083244  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
117083339  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
117083346  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
117083708  SEC62; translocation protein SEC62 [KO:K12275]
117083860  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
117084258  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
117084290  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
117085112  WFS1; wolframin [KO:K14020]
117085254  [KO:K06689] [EC:2.3.2.23]
117085362  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
117085384  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
117085720  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
117085766  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
117085772  SEC24B; LOW QUALITY PROTEIN: protein transport protein Sec24B [KO:K14007]
117085822  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
117085831  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
117085891  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
117086414  [KO:K26501]
117086685  [KO:K03283]
117086689  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
117086698  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
117086963  HSPA1A; LOW QUALITY PROTEIN: heat shock 70 kDa protein 1A [KO:K03283]
117086964  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
117086987  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
117086996  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
117087056  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
117087288  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
117087403  BAG2; BAG family molecular chaperone regulator 2 isoform X1 [KO:K09556]
117087741  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
117087808  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
117087834  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
117088126  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
117089383  ERP29; endoplasmic reticulum resident protein 29 isoform X1 [KO:K09586]
117089480  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
117089519  HSP90B1; endoplasmin [KO:K09487]
117089865  OS9; protein OS-9 isoform X1 [KO:K10088]
117089884  DDIT3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452]
117090255  [KO:K13251]
117090435  [KO:K03094]
117091531  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
117091541  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
117091733  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
117091970  [KO:K07151] [EC:2.4.99.18]
117092410  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
117092884  [KO:K19478]
117093092  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
117093185  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit isoform X1 [KO:K12670]
117093316  FBXO2; F-box only protein 2 [KO:K10099]
117093317  FBXO6; F-box only protein 6 [KO:K10100]
117093342  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
117093478  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
117094183  [KO:K09507]
117094489  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
117094525  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
117094770  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
117094810  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
117095073  UBXN4; UBX domain-containing protein 4 [KO:K24348]
117095178  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
117095846  [KO:K09541]
117096024  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
117096042  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
117096137  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
117096195  [KO:K14021]
117096214  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
117096433  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X1 [KO:K03868] [EC:2.3.2.32]
117096463  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
117096558  DERL3; derlin-3 isoform X1 [KO:K13989]
117096908  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
117096999  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA isoform X1 [KO:K01230] [EC:3.2.1.113]
117097078  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
117097169  SEC63; translocation protein SEC63 homolog [KO:K09540]
117097478  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
117097719  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
117097891  STUB1; E3 ubiquitin-protein ligase CHIP isoform X1 [KO:K09561] [EC:2.3.2.27]
117098029  [KO:K26501]
117098419  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
117098677  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
117098874  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
117098927  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
117099028  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
117099065  BAG1; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555]
117099113  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741]
117099135  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
117099565  LMAN1; protein ERGIC-53 isoform X1 [KO:K10080]
117099723  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
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
tfn00510  N-Glycan biosynthesis
tfn03050  Proteasome
tfn03060  Protein export
tfn04210  Apoptosis
tfn04930  Type II diabetes mellitus
tfn05010  Alzheimer disease
tfn05012  Parkinson disease
tfn05020  Prion disease
KO pathway
ko04141   

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