KEGG   PATHWAY: oma04141
Entry
oma04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Oenanthe melanoleuca (Eastern black-eared wheatear)
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
oma04141  Protein processing in endoplasmic reticulum
oma04141

Other DBs
GO: 0030433 0034976
Organism
Oenanthe melanoleuca (Eastern black-eared wheatear) [GN:oma]
Gene
130248209  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
130258089  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
130249504  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
130249201  SEC61G; protein transport protein Sec61 subunit gamma isoform X1 [KO:K07342]
130257016  SEC62; translocation protein SEC62 [KO:K12275]
130250675  SEC63; translocation protein SEC63 homolog [KO:K09540]
130258419  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
130261237  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
130264179  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
130261559  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
130262546  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
130248951  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
130252515  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
130253027  MAGT1; magnesium transporter protein 1 [KO:K19478]
130252148  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
130253689  TMEM258; transmembrane protein 258 [KO:K26502]
130252094  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
130248302  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
130251627  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
130258635  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
130262580  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
130260132  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
130257028  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
130249790  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
130249824  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
130255805  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
130248583  HSP90B1; endoplasmin [KO:K09487]
130262608  GANAB; LOW QUALITY PROTEIN: neutral alpha-glucosidase AB [KO:K05546] [EC:3.2.1.207]
130258970  CANX; calnexin [KO:K08054]
130257135  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
130262780  CALR; calreticulin [KO:K08057]
130250709  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
130262131  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
130265255  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
130259971  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
130258987  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
130265096  LMAN1; protein ERGIC-53 isoform X1 [KO:K10080]
130251793  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
130258838  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
130255190  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
130258081  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
130252570  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
130254824  SEC31B; protein transport protein Sec31B [KO:K14005]
130251081  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
130253758  SEC23A; protein transport protein Sec23A [KO:K14006]
130252260  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
130252425  SEC24D; protein transport protein Sec24D [KO:K14007]
130258836  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
130254902  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
130255557  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
130256762  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
130258338  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
130261145  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
130256146  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
130260642  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
130249847  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
130251649  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
130259909  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
130249713  TXNDC5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
130254267  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
130251055  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
130265782  OS9; protein OS-9 isoform X1 [KO:K10088]
130251780  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
130249714  SSR1; translocon-associated protein subunit alpha [KO:K13249]
130263179  SSR2; translocon-associated protein subunit beta [KO:K13250]
130256758  SSR3; translocon-associated protein subunit gamma [KO:K13251]
130262839  BCAP31; B-cell receptor-associated protein 31 isoform X1 [KO:K14009]
130250387  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
130250333  DERL1; derlin-1 [KO:K11519]
130259755  DERL3; derlin-3 isoform X1 [KO:K13989]
130266730  derlin-2-like isoform X1 [KO:K13989]
130260895  DERL2; derlin-2 [KO:K13989]
130255397  UBXN4; UBX domain-containing protein 4 [KO:K24348]
130262832  UBXN1; UBX domain-containing protein 1 [KO:K24348]
130250778  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
130264320  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
130261386  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
130253422  SVIP; small VCP/p97-interacting protein [KO:K14014]
130265315  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
130260526  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
130259582  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
130262450  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
130265355  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
130262691  dnaJ homolog subfamily A member 1-like [KO:K09502]
130257731  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
130263062  DNAJB1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
130255334  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
130255191  DNAJB12; dnaJ homolog subfamily B member 12 [KO:K09518]
130250961  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
130261300  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
130248761  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
130251200  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
130253553  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
130252476  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
130249396  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
130250433  BAG1; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555]
130250716  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
130260163  CRYAA; alpha-crystallin A chain [KO:K09541]
130262491  CRYAB; alpha-crystallin B chain [KO:K09542]
130263693  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
130265631  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
130264827  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
130263141  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
130264764  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
130250388  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
130266829  ATXN3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
130261584  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
130252489  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
130253857  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
130255827  NFE2L2; nuclear factor erythroid 2-related factor 2 [KO:K05638]
130261800  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
130265810  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
130249137  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
130256104  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
130252560  WFS1; wolframin isoform X1 [KO:K14020]
130258048  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
130260509  MBTPS2; LOW QUALITY PROTEIN: membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
130259823  XBP1; X-box-binding protein 1 [KO:K09027]
130259312  ERN2; serine/threonine-protein kinase/endoribonuclease IRE2 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
130260397  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
130260219  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
130251886  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
130263048  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
130252708  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
130254720  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
130258632  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
130250076  BAX; apoptosis regulator BAX [KO:K02159]
130263807  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
130251197  CAPN11; calpain-11 isoform X1 [KO:K01367] [EC:3.4.22.52]
130262735  CAPN1; calpain-1 catalytic subunit isoform X1 [KO:K01367] [EC:3.4.22.52]
130250773  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
130248891  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
130251122  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
130261740  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
130260865  UBE2G1; ubiquitin-conjugating enzyme E2 G1 isoform X1 [KO:K10575] [EC:2.3.2.23]
130256601  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
130262927  SYVN1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
130259800  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
130257233  SELENOS; selenoprotein S [KO:K14025]
130253707  SEL1L; protein sel-1 homolog 1 [KO:K14026]
130257802  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
130257661  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
130259119  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
130252132  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
130254699  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
130258657  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
130251513  PRKN; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
130248223  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
130248947  CUL1; cullin-1 [KO:K03347]
130258942  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
130261578  F-box only protein 2-like [KO:K10099]
130261577  F-box only protein 6-like [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
oma00510  N-Glycan biosynthesis
oma03050  Proteasome
oma03060  Protein export
oma04210  Apoptosis
KO pathway
ko04141   
LinkDB

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