KEGG   PATHWAY: gcl04141
Entry
gcl04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Gymnogyps californianus (California condor)
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
gcl04141  Protein processing in endoplasmic reticulum
gcl04141

Other DBs
GO: 0030433 0034976
Organism
Gymnogyps californianus (California condor) [GN:gcl]
Gene
127017099  protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
127021508  protein transport protein Sec61 subunit alpha [KO:K10956]
127013100  protein transport protein Sec61 subunit beta [KO:K09481]
127012781  protein transport protein Sec61 subunit gamma [KO:K07342]
127020363  translocation protein SEC62 isoform X1 [KO:K12275]
127014305  translocation protein SEC63 homolog isoform X1 [KO:K09540]
127021447  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
127023392  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
127026539  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
127024798  dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
127025782  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
127013028  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
127019707  magnesium transporter protein 1 isoform X1 [KO:K19478]
127015668  tumor suppressor candidate 3 [KO:K19478]
127015694  oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
127017396  transmembrane protein 258 [KO:K26502]
127028124  LOW QUALITY PROTEIN: cytoskeleton-associated protein 4 [KO:K13999]
127014071  ribosome-binding protein 1 [KO:K14000]
127021888  LOW QUALITY PROTEIN: nucleotide exchange factor SIL1 [KO:K14001]
127025860  hypoxia up-regulated protein 1 [KO:K09486]
127023758  endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
127019961  dnaJ homolog subfamily B member 11 [KO:K09517]
127012857  dnaJ homolog subfamily C member 1 [KO:K09521]
127013332  dnaJ homolog subfamily C member 3 [KO:K09523]
127018404  dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
127029610  endoplasmin [KO:K09487]
127021863  calnexin isoform X1 [KO:K08054]
127020810  protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
127025702  calreticulin-3 [KO:K08057]
127024961  mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
127014518  mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
127014976  mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
127023863  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
127021906  vesicular integral-membrane protein VIP36 [KO:K10082]
127027409  protein ERGIC-53 [KO:K10080]
127017698  GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
127021926  GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
127021626  protein SEC13 homolog [KO:K14004]
127017974  protein transport protein Sec31B [KO:K14005]
127016210  protein transport protein Sec31A isoform X1 [KO:K14005]
127014440  protein transport protein Sec23B [KO:K14006]
127016499  protein transport protein Sec23A [KO:K14006]
127017607  protein transport protein Sec24C isoform X1 [KO:K14007]
127015389  protein transport protein Sec24D [KO:K14007]
127015493  protein transport protein Sec24B isoform X1 [KO:K14007]
127021845  protein transport protein Sec24A [KO:K14007]
127019881  UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
127027753  UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
127021550  ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
127023238  LOW QUALITY PROTEIN: ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
127019017  ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
127012598  protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
127014299  protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
127022749  endoplasmic reticulum resident protein 29 [KO:K09586]
127012746  thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
127016738  ERO1-like protein alpha isoform X1 [KO:K10950] [EC:1.8.4.-]
127014630  ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
127014787  endoplasmic reticulum lectin 1 [KO:K14008]
127012747  translocon-associated protein subunit alpha isoform X1 [KO:K13249]
127026979  translocon-associated protein subunit beta isoform X1 [KO:K13250]
127027061  translocon-associated protein subunit beta-like [KO:K13250]
127020123  translocon-associated protein subunit gamma [KO:K13251]
127028027  B-cell receptor-associated protein 31 isoform X1 [KO:K14009]
127028458  B-cell receptor-associated protein 31-like [KO:K14009]
127023188  translocating chain-associated membrane protein 1-like 1 isoform X1 [KO:K14010]
127013528  translocating chain-associated membrane protein 1 isoform X1 [KO:K14010]
127029877  derlin-1 isoform X1 [KO:K11519]
127022864  derlin-2-like isoform X1 [KO:K13989]
127018494  UBX domain-containing protein 4 isoform X1 [KO:K24348]
127014147  UBX domain-containing protein 2A isoform X1 [KO:K24349]
127023413  NSFL1 cofactor p47 [KO:K14012]
127016770  small VCP/p97-interacting protein [KO:K14014]
127027123  transitional endoplasmic reticulum ATPase [KO:K13525]
127023912  nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
127022955  ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
127025779  heat shock cognate 71 kDa protein isoform X1 [KO:K03283]
127027127  dnaJ homolog subfamily A member 1 isoform X1 [KO:K09502]
127028756  dnaJ homolog subfamily A member 1-like isoform X1 [KO:K09502]
127020989  dnaJ homolog subfamily A member 2 [KO:K09503]
127018276  dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
127017691  dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
127023145  dnaJ homolog subfamily C member 5 [KO:K09525]
127013541  dnaJ homolog subfamily C member 5B [KO:K09525]
127016915  heat shock protein HSP 90-alpha isoform X1 [KO:K04079]
127014081  heat shock protein HSP 90-beta isoform X1 [KO:K04079]
127021595  heat shock protein 105 kDa isoform X1 [KO:K09485]
127016198  heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
127012968  BAG family molecular chaperone regulator 1 [KO:K09555]
127014823  BAG family molecular chaperone regulator 2 [KO:K09556]
127026431  alpha-crystallin A chain [KO:K09541]
127025873  alpha-crystallin B chain [KO:K09542]
127026140  ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
127027470  phospholipase A-2-activating protein isoform X1 [KO:K14018]
127027282  UV excision repair protein RAD23 homolog B [KO:K10839]
127025842  ubiquilin-2-like [KO:K04523]
127027031  ubiquilin-4 [KO:K04523]
127027312  ubiquilin-1 isoform X1 [KO:K04523]
127027457  ubiquilin-1-like isoform X1 [KO:K04523]
127013064  LOW QUALITY PROTEIN: peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
127016408  ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
127024709  ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
127015534  eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
127016983  eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
127018157  nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
127028956  cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
127028534  DNA damage-inducible transcript 3 protein [KO:K04452]
127013629  apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
127019162  cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
127015742  wolframin [KO:K14020]
127020932  membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
127023684  membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
127023026  LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
127022294  serine/threonine-protein kinase/endoribonuclease IRE2 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
127024121  serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
127023695  TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
127014170  LOW QUALITY PROTEIN: mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
127022185  mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
127017578  mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
127015438  mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
127026190  bcl-2 homologous antagonist/killer [KO:K14021]
127014122  LOW QUALITY PROTEIN: calpain-11 [KO:K01367] [EC:3.4.22.52]
127029143  calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
127015289  calpain-2 catalytic subunit isoform X1 [KO:K03853] [EC:3.4.22.53]
127012548  E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
127014655  ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
127024689  ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
127024428  ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
127020272  ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
127029152  E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
127023050  E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
127020499  selenoprotein S [KO:K14025]
127016554  protein sel-1 homolog 1 isoform X1 [KO:K14026]
127021035  homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
127021012  E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
127017638  ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
127016020  ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
127021811  ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
127014119  E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
127022521  E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
127012844  cullin-1 isoform X1 [KO:K03347]
127021889  S-phase kinase-associated protein 1 [KO:K03094]
127024652  F-box only protein 2-like [KO:K10099]
127024651  F-box only protein 6-like [KO:K10100]
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
gcl00510  N-Glycan biosynthesis
gcl03050  Proteasome
gcl03060  Protein export
gcl04210  Apoptosis
KO pathway
ko04141   
LinkDB

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