KEGG   PATHWAY: oor04141
Entry
oor04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Orcinus orca (killer whale)
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
oor04141  Protein processing in endoplasmic reticulum
oor04141

Other DBs
GO: 0030433 0034976
Organism
Orcinus orca (killer whale) [GN:oor]
Gene
101271372  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
101284382  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
101288059  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
101275644  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
117201101  protein transport protein Sec61 subunit gamma-like [KO:K07342]
101288732  SEC62; translocation protein SEC62 [KO:K12275]
101289771  SEC63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
101283135  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
101273868  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X9 [KO:K12667]
101286583  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
101287900  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
101289840  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
101269582  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
101275376  MAGT1; magnesium transporter protein 1 isoform X1 [KO:K19478]
101272277  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
101286793  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
101275800  oligosaccharyltransferase complex subunit OSTC-like [KO:K26501]
101275048  TMEM258; transmembrane protein 258 [KO:K26502]
101274685  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
101284321  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
101277188  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
101269667  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
101273204  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
101289151  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
101283008  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
101270242  DNAJC1; dnaJ homolog subfamily C member 1 isoform X2 [KO:K09521]
101271276  DNAJC3; dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
101272335  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
101289801  HSP90B1; endoplasmin [KO:K09487]
101280187  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
101287242  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
101285652  CANX; calnexin isoform X1 [KO:K08054]
101282830  PDIA3; protein disulfide-isomerase A3 isoform X1 [KO:K08056] [EC:5.3.4.1]
101279584  CALR; calreticulin [KO:K08057]
101277960  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA isoform X1 [KO:K01230] [EC:3.2.1.113]
101283566  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
101282517  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
101275150  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741] [EC:3.2.1.209]
101278381  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
101274095  LMAN1; protein ERGIC-53 isoform X1 [KO:K10080]
101270697  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
101287842  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
101269334  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
101273873  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
101285891  SEC31A; protein transport protein Sec31A isoform X9 [KO:K14005]
101284575  LOW QUALITY PROTEIN: protein transport protein Sec31B [KO:K14005]
101272597  SEC23A; protein transport protein Sec23A [KO:K14006]
101279893  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
101287586  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
101285005  SEC24C; protein transport protein Sec24C isoform X2 [KO:K14007]
101290181  SEC24D; protein transport protein Sec24D isoform X2 [KO:K14007]
101285686  SEC24B; protein transport protein Sec24B isoform X3 [KO:K14007]
101270795  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X2 [KO:K11718] [EC:2.4.1.-]
101273551  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
101283846  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
101283160  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
101287402  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X4 [KO:K10086]
101274850  P4HB; protein disulfide-isomerase isoform X2 [KO:K09580] [EC:5.3.4.1]
101281871  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
101269475  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
101286315  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
101280780  thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
101284444  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
101281527  ERO1A; ERO1-like protein alpha isoform X1 [KO:K10950] [EC:1.8.4.-]
101272510  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
101281844  OS9; protein OS-9 isoform X2 [KO:K10088]
105748937  ERLEC1; endoplasmic reticulum lectin 1 isoform X5 [KO:K14008]
101285734  SSR1; translocon-associated protein subunit alpha [KO:K13249]
101284887  SSR2; translocon-associated protein subunit beta [KO:K13250]
101272638  SSR3; translocon-associated protein subunit gamma isoform X1 [KO:K13251]
101277902  SSR4; translocon-associated protein subunit delta isoform X3 [KO:K04571]
101276077  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
101273408  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
101269517  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
101281053  DERL1; derlin-1 isoform X2 [KO:K11519]
101289140  DERL2; derlin-2 isoform X1 [KO:K13989]
101275452  DERL3; derlin-3 isoform X1 [KO:K13989]
101269767  UBXN4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
101281628  UBXN1; UBX domain-containing protein 1 [KO:K24348]
101280642  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
101290320  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
101287066  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
101280909  NSFL1C; NSFL1 cofactor p47 isoform X2 [KO:K14012]
101281129  SVIP; small VCP/p97-interacting protein [KO:K14014]
117200709  small VCP/p97-interacting protein [KO:K14014]
101289076  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
101272195  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
101286387  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
101278046  heat shock cognate 71 kDa protein [KO:K03283]
101283817  HSPA1B; heat shock 70 kDa protein 1B [KO:K03283]
101284066  heat shock 70 kDa protein 1B [KO:K03283]
101284311  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
101282770  LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
101284349  heat shock cognate 71 kDa protein-like [KO:K03283]
125963979  LOW QUALITY PROTEIN: heat shock 70 kDa protein 6-like [KO:K03283]
125965152  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
101284841  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
101270141  LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 1-like [KO:K09502]
101288031  DNAJA2; dnaJ homolog subfamily A member 2 isoform X1 [KO:K09503]
101270063  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
101283365  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
101271363  DNAJB12; dnaJ homolog subfamily B member 12 isoform X2 [KO:K09518]
101275505  DNAJC5G; LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 5G [KO:K09525]
101277305  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
101270717  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
101285036  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
101278395  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
101271249  LOW QUALITY PROTEIN: heat shock protein HSP 90-beta-like [KO:K04079]
101278454  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
101279278  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
101277692  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
101273607  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
101275047  HSPBP1; hsp70-binding protein 1 [KO:K09562]
101282191  CRYAA; alpha-crystallin A chain [KO:K09541]
101274522  CRYAB; alpha-crystallin B chain [KO:K09542]
101270913  YOD1; ubiquitin thioesterase OTU1 isoform X1 [KO:K13719] [EC:3.1.2.-]
101279401  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
101275848  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
101279159  RAD23A; UV excision repair protein RAD23 homolog A isoform X2 [KO:K10839]
101285249  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
101273560  UBQLN3; ubiquilin-3 [KO:K04523]
101284472  UBQLN4; ubiquilin-4 isoform X3 [KO:K04523]
101277544  UBQLN2; ubiquilin-2 [KO:K04523]
101269797  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
101289514  ATXN3; ataxin-3 isoform X3 [KO:K11863] [EC:3.4.22.-]
101280315  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
101282451  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
101281288  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
101281809  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X4 [KO:K05638]
101283438  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
101289075  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
101278090  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
101276750  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
101284204  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
101273605  ATF6B; LOW QUALITY PROTEIN: cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
101281370  WFS1; wolframin [KO:K14020]
101282350  MBTPS1; membrane-bound transcription factor site-1 protease isoform X2 [KO:K08653] [EC:3.4.21.112]
101272405  MBTPS2; membrane-bound transcription factor site-2 protease isoform X3 [KO:K07765] [EC:3.4.24.85]
101288279  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
101288419  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
101281303  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
101277232  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
101288079  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X3 [KO:K04431] [EC:2.7.12.2]
101287701  MAPK9; mitogen-activated protein kinase 9 isoform X5 [KO:K04440] [EC:2.7.11.24]
101277656  MAPK10; mitogen-activated protein kinase 10 isoform X4 [KO:K04440] [EC:2.7.11.24]
101281228  MAPK8; mitogen-activated protein kinase 8 isoform X2 [KO:K04440] [EC:2.7.11.24]
101285349  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
101281395  BAK1; bcl-2 homologous antagonist/killer isoform X2 [KO:K14021]
101287191  CAPN1; calpain-1 catalytic subunit isoform X2 [KO:K01367] [EC:3.4.22.52]
101270285  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
101272972  caspase-12 isoform X1 [KO:K04741] [EC:3.4.22.-]
101283845  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X3 [KO:K10661] [EC:2.3.2.27]
101286765  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
101277012  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X2 [KO:K04554] [EC:2.3.2.23]
101272076  UBE2G1; ubiquitin-conjugating enzyme E2 G1 isoform X2 [KO:K10575] [EC:2.3.2.23]
101285835  ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
101285399  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X3 [KO:K04555] [EC:2.3.2.23]
101286505  SYVN1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
101277249  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
101278895  SELENOS; selenoprotein S isoform X1 [KO:K14025]
101276860  SEL1L2; protein sel-1 homolog 2 isoform X2 [KO:K14026]
101277717  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
101277966  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
101285163  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
101289637  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
101275624  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X2 [KO:K06689] [EC:2.3.2.23]
101281045  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X2 [KO:K06689] [EC:2.3.2.23]
101280529  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X4 [KO:K06689] [EC:2.3.2.23]
101275328  ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
125963065  ubiquitin-conjugating enzyme E2 D3-like [KO:K06689] [EC:2.3.2.23]
101287254  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
101289349  RING-box protein 1-like [KO:K03868] [EC:2.3.2.32]
101280362  CUL1; cullin-1 [KO:K03347]
101276725  cullin-1-like [KO:K03347]
101289355  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
101285269  FBXO2; F-box only protein 2 [KO:K10099]
101285947  FBXO6; F-box only protein 6 [KO:K10100]
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
oor00510  N-Glycan biosynthesis
oor03050  Proteasome
oor03060  Protein export
oor04210  Apoptosis
oor04930  Type II diabetes mellitus
oor05010  Alzheimer disease
oor05012  Parkinson disease
oor05020  Prion disease
KO pathway
ko04141   
LinkDB

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