KEGG   PATHWAY: rbb04141
Entry
rbb04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Rhinopithecus bieti (black snub-nosed 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
rbb04141  Protein processing in endoplasmic reticulum
rbb04141

Other DBs
GO: 0030433 0034976
Organism
Rhinopithecus bieti (black snub-nosed monkey) [GN:rbb]
Gene
108512112  UBXN6; UBX domain-containing protein 6 [KO:K14011]
108512121  ERP29; endoplasmic reticulum resident protein 29 isoform X1 [KO:K09586]
108512292  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
108512463  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
108512696  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
108513145  SEC62; translocation protein SEC62 [KO:K12275]
108513174  [KO:K04741] [EC:3.4.22.-]
108513689  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
108514389  DERL2; derlin-2 isoform X1 [KO:K13989]
108514775  DNAJB1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
108515301  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
108515459  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
108515541  SSR2; translocon-associated protein subunit beta [KO:K13250]
108515542  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
108516259  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
108516435  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
108516559  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
108516849  SEC24C; protein transport protein Sec24C [KO:K14007]
108516893  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
108516971  SEC31A; LOW QUALITY PROTEIN: protein transport protein Sec31A [KO:K14005]
108517254  SSR3; translocon-associated protein subunit gamma [KO:K13251]
108517508  [KO:K04440] [EC:2.7.11.24]
108517565  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
108517777  DERL1; derlin-1 [KO:K11519]
108518016  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
108518240  DNAJA1; dnaJ homolog subfamily A member 1 isoform X1 [KO:K09502]
108518377  UFD1L; ubiquitin fusion degradation protein 1 homolog isoform X1 [KO:K14016]
108518462  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
108518634  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
108518882  EIF2AK3; LOW QUALITY PROTEIN: eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
108519113  ATXN3; LOW QUALITY PROTEIN: ataxin-3 [KO:K11863] [EC:3.4.22.-]
108519175  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
108519219  [KO:K10956]
108519428  STUB1; E3 ubiquitin-protein ligase CHIP isoform X1 [KO:K09561] [EC:2.3.2.27]
108519685  DDIT3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452]
108519784  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
108519829  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
108519893  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
108519898  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
108519916  MAGT1; magnesium transporter protein 1 [KO:K19478]
108519923  DNAJC3; dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
108520227  [KO:K03283]
108520228  [KO:K03283]
108520463  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
108520713  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
108521141  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
108521220  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
108521293  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
108522042  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
108522196  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
108522301  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
108522304  CUL1; cullin-1 [KO:K03347]
108522926  VIMP; selenoprotein S isoform X1 [KO:K14025]
108522968  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
108523186  SEC23B; protein transport protein Sec23B [KO:K14006]
108523361  CANX; calnexin [KO:K08054]
108523574  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
108523624  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
108523638  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
108523901  [KO:K11863] [EC:3.4.22.-]
108524399  OS9; protein OS-9 isoform X1 [KO:K10088]
108524470  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
108524828  HSPH1; LOW QUALITY PROTEIN: heat shock protein 105 kDa [KO:K09485]
108525569  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
108526412  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
108526453  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
108526546  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
108526611  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
108526614  DNAJC10; LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
108526833  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
108527063  BAG2; BAG family molecular chaperone regulator 2 isoform X1 [KO:K09556]
108527136  NFE2L2; LOW QUALITY PROTEIN: nuclear factor erythroid 2-related factor 2 [KO:K05638]
108527616  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
108527842  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
108527914  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
108527984  PARK2; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
108528145  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
108528248  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
108528465  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
108528637  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
108528732  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
108528791  [KO:K03283]
108528804  DNAJC5B; dnaJ homolog subfamily C member 5B isoform X1 [KO:K09525]
108528878  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
108529545  [KO:K14007]
108529547  SAR1B; GTP-binding protein SAR1b isoform X1 [KO:K07953] [EC:3.6.5.-]
108529558  SKP1; LOW QUALITY PROTEIN: S-phase kinase-associated protein 1 [KO:K03094]
108529602  [KO:K09541]
108529738  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA isoform X1 [KO:K01230] [EC:3.2.1.113]
108529850  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
108530078  TRAM1; LOW QUALITY PROTEIN: translocating chain-associated membrane protein 1 [KO:K14010]
108530350  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
108530813  SEL1L; protein sel-1 homolog 1 [KO:K14026]
108530851  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
108531361  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
108531477  LMAN1L; protein ERGIC-53-like isoform X1 [KO:K10081]
108531677  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
108531812  [KO:K08057]
108531954  HSPA8; LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein [KO:K03283]
108532305  MBTPS2; LOW QUALITY PROTEIN: membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
108532549  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
108532559  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
108532632  UBE2J1; LOW QUALITY PROTEIN: ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
108532703  PREB; prolactin regulatory element-binding protein [KO:K14003]
108532710  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
108532810  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
108533442  HSP90AB1; LOW QUALITY PROTEIN: heat shock protein HSP 90-beta [KO:K04079]
108533575  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
108533581  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
108533604  TMEM258; transmembrane protein 258 [KO:K26502]
108533712  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
108533818  SEC63; translocation protein SEC63 homolog [KO:K09540]
108533840  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
108533865  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
108534108  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
108534121  NPLOC4; LOW QUALITY PROTEIN: nuclear protein localization protein 4 homolog [KO:K14015]
108534313  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
108534322  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
108534524  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
108534646  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
108534777  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
108535079  SSR4; translocon-associated protein subunit delta [KO:K04571]
108535082  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
108535134  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
108535173  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
108535324  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
108536091  UBQLN2; ubiquilin-2 [KO:K04523]
108536259  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
108536457  CRYAB; alpha-crystallin B chain isoform X1 [KO:K09542]
108537061  [KO:K04079]
108537393  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
108537478  SEC61A1; LOW QUALITY PROTEIN: protein transport protein Sec61 subunit alpha [KO:K10956]
108537517  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
108537794  MAPK8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
108538528  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
108538567  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
108538799  WFS1; LOW QUALITY PROTEIN: wolframin [KO:K14020]
108538969  UBQLN3; ubiquilin-3 [KO:K04523]
108539373  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
108539375  FBXO2; F-box only protein 2 [KO:K10099]
108539377  FBXO6; F-box only protein 6 [KO:K10100]
108539634  UBXN4; UBX domain-containing protein 4 [KO:K24348]
108539683  MARCH6; E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
108539825  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
108539838  [KO:K04571]
108540426  DERL3; derlin-3 isoform X1 [KO:K13989]
108540597  [KO:K03094]
108540738  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
108541019  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
108541064  LMAN1; protein ERGIC-53 [KO:K10080]
108541686  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
108541796  SVIP; LOW QUALITY PROTEIN: small VCP/p97-interacting protein [KO:K14014]
108542298  HSP90AA1; LOW QUALITY PROTEIN: heat shock protein HSP 90-alpha [KO:K04079]
108542335  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
108542527  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
108542583  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
108542584  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
108542887  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741]
108542901  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
108543029  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
108543075  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
108543228  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
108543285  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
108544010  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
108544095  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
108544097  CALR; calreticulin [KO:K08057]
108544203  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
108544250  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
108544251  HSP90B1; LOW QUALITY PROTEIN: endoplasmin [KO:K09487]
108544295  [KO:K03094]
108544361  SEC23A; protein transport protein Sec23A isoform X1 [KO:K14006]
108544403  RRBP1; LOW QUALITY PROTEIN: ribosome-binding protein 1 [KO:K14000]
108544541  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
108544555  [KO:K14004]
108544779  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
108544961  [KO:K11863] [EC:3.4.22.-]
Compound
C00076  Calcium cation
G00009  
G00010  
G00011  
G00012  
G10694  
Reference
  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
  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
  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
  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
  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
rbb00510  N-Glycan biosynthesis
rbb03050  Proteasome
rbb03060  Protein export
rbb04210  Apoptosis
rbb04930  Type II diabetes mellitus
rbb05010  Alzheimer disease
rbb05012  Parkinson disease
rbb05020  Prion disease
KO pathway
ko04141   

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