KEGG   PATHWAY: fga04141
Entry
fga04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Fulmarus glacialis (Northern fulmar)
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
fga04141  Protein processing in endoplasmic reticulum
fga04141

Other DBs
GO: 0030433 0034976
Organism
Fulmarus glacialis (Northern fulmar) [GN:fga]
Gene
104076528  SEC61A1; protein transport protein Sec61 subunit alpha isoform 1 [KO:K10956]
104070113  SEC61A2; protein transport protein Sec61 subunit alpha isoform 2 [KO:K10956]
104072066  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
104071590  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
104071227  SEC62; translocation protein SEC62 [KO:K12275]
104073936  SEC63; translocation protein SEC63 homolog [KO:K09540]
104072717  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
104070244  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
104076652  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
104069514  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
104078832  LOW QUALITY PROTEIN: dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B-like [KO:K07151] [EC:2.4.99.18]
104077147  MAGT1; magnesium transporter protein 1 [KO:K19478]
104081036  TUSC3; tumor suppressor candidate 3 [KO:K19478]
104074758  oligosaccharyltransferase complex subunit ostc [KO:K26501]
104072113  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
104073551  RRBP1; ribosome-binding protein 1 [KO:K14000]
104074174  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
104072491  HYOU1; LOW QUALITY PROTEIN: hypoxia up-regulated protein 1 [KO:K09486]
104070261  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
104078141  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
104080701  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
104077832  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
104069751  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
104069919  HSP90B1; endoplasmin [KO:K09487]
104083951  LOW QUALITY PROTEIN: calnexin-like [KO:K08054]
104077809  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
104081860  LOW QUALITY PROTEIN: calreticulin-like [KO:K08057]
104077776  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
104081354  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like [KO:K23741] [EC:3.2.1.209]
104070865  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
104070544  LMAN1; protein ERGIC-53 [KO:K10080]
104071658  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
104071464  GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
104071135  SEC13; protein SEC13 homolog [KO:K14004]
104077530  SEC31B; LOW QUALITY PROTEIN: protein transport protein Sec31B [KO:K14005]
104070208  SEC31A; protein transport protein Sec31A [KO:K14005]
104077640  SEC23A; protein transport protein Sec23A [KO:K14006]
104083162  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
104077330  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
104073421  SEC24B; LOW QUALITY PROTEIN: protein transport protein Sec24B [KO:K14007]
104074757  SEC24A; protein transport protein Sec24A [KO:K14007]
104069101  SEC24D; protein transport protein Sec24D [KO:K14007]
104080597  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
104081420  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
104073672  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
104079510  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
104070515  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
104079607  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
104083780  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
104071761  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
104070671  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
104079632  ERO1L; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
104070073  ERO1LB; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
104083426  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
104071411  SSR1; translocon-associated protein subunit alpha [KO:K13249]
104082466  LOW QUALITY PROTEIN: translocon-associated protein subunit beta-like [KO:K13250]
104071673  translocon-associated protein subunit beta [KO:K13250]
104071440  SSR3; translocon-associated protein subunit gamma [KO:K13251]
104070696  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
104078208  translocating chain-associated membrane protein 1-like 1 [KO:K14010]
104074532  DERL1; derlin-1 [KO:K11519]
104081282  DERL3; derlin-3 [KO:K13989]
104076712  DERL2; derlin-2 [KO:K13989]
104076579  UBXN4; UBX domain-containing protein 4 [KO:K24348]
104082550  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
104073919  UBXN6; UBX domain-containing protein 6 [KO:K14011]
104070541  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
104077170  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
104073190  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
104081990  UFD1L; ubiquitin fusion degradation protein 1 homolog [KO:K14016]
104080404  HSPA8; heat shock cognate 71 kDa protein isoform X1 [KO:K03283]
104080111  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
104083907  DNAJA2; LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 2 [KO:K09503]
104073972  DNAJB2; dnaJ homolog subfamily B member 2 [KO:K09508]
104083059  DNAJB12; dnaJ homolog subfamily B member 12 [KO:K09518]
104072017  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
104071277  LOW QUALITY PROTEIN: cysteine string protein-like [KO:K09525]
104074664  HSP90AB1; heat shock protein HSP 90-beta isoform X1 [KO:K04079]
104080193  HSP90AA1; heat shock protein HSP 90-alpha isoform X1 [KO:K04079]
104079495  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
104079820  heat shock protein 105 kDa-like [KO:K09485]
104070973  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
104072206  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
104073222  alpha-crystallin A chain [KO:K09541]
104072171  CRYAB; alpha-crystallin B chain [KO:K09542]
104072118  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
104072535  PLAA; phospholipase A-2-activating protein [KO:K14018]
104079437  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
104083387  ubiquilin-1-like [KO:K04523]
104071499  ubiquilin-1-like [KO:K04523]
104074959  UBQLN4; ubiquilin-4 [KO:K04523]
104080279  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
104070217  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
104072609  UBE4B; ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
104077426  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
104083631  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 isoform X1 [KO:K03237]
104070699  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
104081561  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
104074711  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
104080197  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
104083106  WFS1; LOW QUALITY PROTEIN: wolframin [KO:K14020]
104073097  MBTPS1; LOW QUALITY PROTEIN: membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
104071663  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
104072589  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
104080806  serine/threonine-protein kinase/endoribonuclease IRE1-like [KO:K08852] [EC:2.7.11.1 3.1.26.-]
104083588  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
104076852  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
104075665  MAPK8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
104080968  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
104078543  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
104074592  LOW QUALITY PROTEIN: calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
104081233  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
104072882  MARCH6; E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
104079998  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
104074822  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
104071326  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
104080667  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
104071270  RNF185; E3 ubiquitin-protein ligase RNF185 isoform X1 [KO:K10666] [EC:2.3.2.27]
104078621  VIMP; selenoprotein S [KO:K14025]
104076109  SEL1L; protein sel-1 homolog 1 [KO:K14026]
104078056  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
104069616  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
104075989  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
104072561  ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
104069162  ubiquitin-conjugating enzyme E2 D2-like [KO:K06689] [EC:2.3.2.23]
104071556  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
104079810  LOW QUALITY PROTEIN: ubiquitin-conjugating enzyme E2 D2-like [KO:K06689] [EC:2.3.2.23]
104079903  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
104083690  CUL1; cullin-1 [KO:K03347]
104071429  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
104075284  FBXO2; F-box only protein 2 [KO:K10099]
104070990  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
fga00510  N-Glycan biosynthesis
fga03050  Proteasome
fga03060  Protein export
fga04210  Apoptosis
KO pathway
ko04141   
LinkDB

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