KEGG   PATHWAY: ray04141
Entry
ray04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Rousettus aegyptiacus (Egyptian rousette)
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
ray04141  Protein processing in endoplasmic reticulum
ray04141

Other DBs
GO: 0030433 0034976
Organism
Rousettus aegyptiacus (Egyptian rousette) [GN:ray]
Gene
107497052  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 isoform X1 [KO:K12666]
107497502  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
107497519  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
107498124  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
107498209  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
107498213  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
107498352  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
107498439  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
107498457  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
107498469  [KO:K03283]
107498470  [KO:K03283]
107498471  [KO:K03283]
107498539  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
107498831  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
107498869  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
107498937  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
107498969  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
107499658  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
107499772  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
107499908  DNAJC1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
107500042  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
107500535  CUL1; cullin-1 isoform X1 [KO:K03347]
107500551  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
107500591  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
107500804  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
107500816  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
107501057  [KO:K04523]
107501143  HSP90B1; endoplasmin [KO:K09487]
107501200  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
107501244  DERL3; derlin-3 isoform X1 [KO:K13989]
107501318  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
107501370  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
107501473  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
107501502  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
107501503  OS9; protein OS-9 isoform X1 [KO:K10088]
107501577  UBXN1; UBX domain-containing protein 1 [KO:K24348]
107501595  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
107501602  TMEM258; transmembrane protein 258 [KO:K26502]
107501685  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
107501913  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X2 [KO:K14027]
107501998  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
107502023  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
107502134  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
107502163  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
107502472  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
107502480  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
107502524  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
107502624  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
107502728  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
107502754  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
107502806  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
107502827  [KO:K13525]
107502936  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
107503081  TRAF2; TNF receptor-associated factor 2 isoform X3 [KO:K03173] [EC:2.3.2.27]
107503110  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X2 [KO:K23741]
107503228  DERL1; derlin-1 [KO:K11519]
107503258  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
107503644  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
107503655  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
107503848  MAGT1; magnesium transporter protein 1 [KO:K19478]
107503908  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
107503915  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
107504150  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
107504291  [KO:K04741] [EC:3.4.22.-]
107504300  SEL1L2; protein sel-1 homolog 2 isoform X2 [KO:K14026]
107504499  DERL2; derlin-2 isoform X1 [KO:K13989]
107504702  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
107504705  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
107504854  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
107505074  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
107505458  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
107505806  UBXN8; UBX domain-containing protein 8 [KO:K24351]
107505881  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
107506115  STUB1; E3 ubiquitin-protein ligase CHIP isoform X2 [KO:K09561] [EC:2.3.2.27]
107506635  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
107506699  SEL1L; protein sel-1 homolog 1 [KO:K14026]
107506799  SKP1; S-phase kinase-associated protein 1 isoform X2 [KO:K03094]
107506806  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
107506807  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
107507194  [KO:K07765] [EC:3.4.24.85]
107507200  TXNDC5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
107507400  [KO:K14014]
107507496  SEC23A; protein transport protein Sec23A [KO:K14006]
107507587  SSR3; translocon-associated protein subunit gamma [KO:K13251]
107507665  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
107507921  HYOU1; hypoxia up-regulated protein 1 isoform X3 [KO:K09486]
107508661  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
107508788  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
107508828  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
107508833  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
107509910  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
107510192  CRYAA; alpha-crystallin A chain [KO:K09541]
107510531  LMAN1L; protein ERGIC-53-like [KO:K10081]
107510708  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
107510736  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
107510886  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
107510893  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
107510959  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
107511210  UBXN4; UBX domain-containing protein 4 [KO:K24348]
107511236  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
107511305  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
107511365  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
107511614  SEC23B; protein transport protein Sec23B [KO:K14006]
107511826  DNAJC5; dnaJ homolog subfamily C member 5 isoform X1 [KO:K09525]
107511968  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
107512264  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
107512317  CANX; calnexin isoform X2 [KO:K08054]
107512328  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
107512376  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
107512498  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
107512510  SEC62; translocation protein SEC62 [KO:K12275]
107512601  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
107512922  NSFL1C; NSFL1 cofactor p47 isoform X2 [KO:K14012]
107512973  CRYAB; alpha-crystallin B chain [KO:K09542]
107513042  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
107513116  [KO:K10100]
107513117  FBXO6; F-box only protein 6 isoform X1 [KO:K10100]
107513118  FBXO2; F-box only protein 2 [KO:K10099]
107513267  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
107513285  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
107514307  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
107514669  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
107514810  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
107514829  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
107515101  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
107515226  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
107515392  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
107515413  HSPBP1; hsp70-binding protein 1 [KO:K09562]
107515782  SELENOS; selenoprotein S [KO:K14025]
107516199  LMAN1; protein ERGIC-53 [KO:K10080]
107516470  SEC31A; protein transport protein Sec31A isoform X8 [KO:K14005]
107516488  PLAA; phospholipase A-2-activating protein [KO:K14018]
107516766  DNAJB2; dnaJ homolog subfamily B member 2 [KO:K09508]
107516883  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X2 [KO:K11718] [EC:2.4.1.-]
107517549  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
107517651  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X2 [KO:K11718] [EC:2.4.1.-]
107517652  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
107517894  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
107518491  SEC63; translocation protein SEC63 homolog [KO:K09540]
107518706  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
107518829  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
107518910  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
107519034  WFS1; wolframin [KO:K14020]
107519418  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
107519440  CALR; calreticulin [KO:K08057]
107519441  RAD23A; UV excision repair protein RAD23 homolog A [KO:K10839]
107519523  DNAJB12; dnaJ homolog subfamily B member 12 isoform X2 [KO:K09518]
107519712  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
107519926  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
107520051  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
107520156  UBE4B; ubiquitin conjugation factor E4 B isoform X2 [KO:K10597] [EC:2.3.2.27]
107520209  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
107520276  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
107520456  [KO:K26502]
107520486  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
107520751  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
107520796  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
107521028  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
107521291  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
107521339  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
107521381  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
107521443  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
107521518  UBQLN4; ubiquilin-4 [KO:K04523]
107521527  SSR2; translocon-associated protein subunit beta [KO:K13250]
107521568  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
107521846  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
107521927  UBQLN2; ubiquilin-2 [KO:K04523]
118607827  [KO:K06689] [EC:2.3.2.23]
118613579  [KO:K14016]
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
ray00510  N-Glycan biosynthesis
ray03050  Proteasome
ray03060  Protein export
ray04210  Apoptosis
ray04930  Type II diabetes mellitus
ray05010  Alzheimer disease
ray05012  Parkinson disease
ray05020  Prion disease
KO pathway
ko04141   

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