KEGG   PATHWAY: hmg04141
Entry
hmg04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Hydra vulgaris (swiftwater hydra)
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
hmg04141  Protein processing in endoplasmic reticulum
hmg04141

Other DBs
GO: 0030433 0034976
Organism
Hydra vulgaris (swiftwater hydra) [GN:hmg]
Gene
100200475  protein transport protein Sec61 subunit alpha [KO:K10956]
100205562  protein transport protein Sec61 subunit beta [KO:K09481]
100213386  translocation protein SEC62 [KO:K12275]
100215612  translocation protein SEC63 homolog [KO:K09540]
100197385  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
100208828  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
101238201  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit dad1 [KO:K12668]
100204358  dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
100202029  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
100205648  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
100211270  tumor suppressor candidate 3 [KO:K19478]
101235901  oligosaccharyltransferase complex subunit ostc [KO:K26501]
100201768  transmembrane protein 258 [KO:K26502]
100204917  mannosyl-oligosaccharide glucosidase GCS1 [KO:K01228] [EC:3.2.1.106]
100207222  nucleotide exchange factor SIL1 [KO:K14001]
100206170  hypoxia up-regulated protein 1 [KO:K09486]
100200137  endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
100209076  dnaJ homolog subfamily B member 11 [KO:K09517]
100202352  dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
100208071  dnaJ homolog subfamily C member 3 [KO:K09523]
100206669  dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
100215003  endoplasmin [KO:K09487]
100215131  neutral alpha-glucosidase AB [KO:K05546] [EC:3.2.1.207]
100205837  neutral alpha-glucosidase C isoform X1 [KO:K05546] [EC:3.2.1.207]
100203932  glucosidase 2 subunit beta isoform X1 [KO:K08288]
100210123  calnexin [KO:K08054]
100203161  protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
100203010  calreticulin isoform X1 [KO:K08057]
100211957  mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
100198486  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
100213998  vesicular integral-membrane protein VIP36 [KO:K10082]
100211467  protein ERGIC-53 [KO:K10080]
100213545  prolactin regulatory element-binding protein [KO:K14003]
100207015  GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
100197954  protein transport protein Sec31A isoform X1 [KO:K14005]
100210272  protein transport protein Sec23B [KO:K14006]
100203783  protein transport protein Sec24A [KO:K14007]
100205491  protein transport protein Sec24D [KO:K14007]
100200765  UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
100208124  ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
100205423  ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
100197565  ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
100204811  protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
100203193  protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
100198247  probable protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
100199495  protein disulfide isomerase-like 2-2 isoform X1 [KO:K09584] [EC:5.3.4.1]
100208034  thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
100212409  ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
100215933  putative leucine-rich repeat-containing protein DDB_G0290503 isoform X1 [KO:K10088]
100213646  endoplasmic reticulum lectin 1 [KO:K14008]
100204271  translocon-associated protein subunit alpha [KO:K13249]
100206362  translocon-associated protein subunit beta [KO:K13250]
100214875  translocon-associated protein subunit gamma [KO:K13251]
100206203  translocon-associated protein subunit delta [KO:K04571]
101235994  B-cell receptor-associated protein 31 [KO:K14009]
100200891  translocating chain-associated membrane protein 1-like 1 [KO:K14010]
100205732  derlin-1 [KO:K11519]
100206953  derlin-2 [KO:K13989]
100197711  UBX domain-containing protein 4 [KO:K24348]
101240216  UBX domain-containing protein 1-B [KO:K24348]
100208476  UBX domain-containing protein 6 [KO:K14011]
100209020  NSFL1 cofactor p47 [KO:K14012]
100205318  transitional endoplasmic reticulum ATPase [KO:K13525]
100212704  nuclear protein localization protein 4 homolog [KO:K14015]
100203935  ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
100203304  heat shock 70 kDa protein [KO:K03283]
100202286  heat shock 70 kDa protein-like [KO:K03283]
105844776  heat shock 70 kDa protein-like [KO:K03283]
124807918  heat shock 70 kDa protein-like [KO:K03283]
124807919  heat shock 70 kDa protein-like [KO:K03283]
124807971  heat shock 70 kDa protein-like [KO:K03283]
124807972  heat shock 70 kDa protein-like [KO:K03283]
124816626  heat shock 70 kDa protein-like [KO:K03283]
124816818  heat shock 70 kDa protein-like [KO:K03283]
100201285  dnaJ homolog subfamily A member 1 [KO:K09502]
100207309  dnaJ homolog subfamily B member 12 [KO:K09518]
100196995  dnaJ homolog subfamily C member 5 isoform X2 [KO:K09525]
100199389  heat shock protein HSP 90-alpha [KO:K04079]
101235897  heat shock protein HSP 90-alpha [KO:K04079]
100202835  97 kDa heat shock protein [KO:K09485]
100199450  BAG family molecular chaperone regulator 1 [KO:K09555]
100203178  hsp70-binding protein 1 isoform X1 [KO:K09562]
100199246  alpha-crystallin B chain-like [KO:K09542]
100214931  heat shock protein hsp-16.2-like [KO:K09542]
105843824  uncharacterized protein LOC105843824 [KO:K09542]
100206652  ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
100202945  phospholipase A-2-activating protein [KO:K14018]
100201209  UV excision repair protein RAD23 homolog B [KO:K10839]
100211830  ubiquilin-1-like isoform X1 [KO:K04523]
100199207  ataxin-3 [KO:K11863] [EC:3.4.22.-]
100206462  ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
101239642  eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
100199407  eukaryotic translation initiation factor 2 subunit 1-like [KO:K03237]
100197237  uncharacterized protein LOC100197237 [KO:K04374]
100215239  membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
100215228  membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
100198653  X-box-binding protein 1 [KO:K09027]
100198901  serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
100213781  TNF receptor-associated factor 5 [KO:K03173] [EC:2.3.2.27]
100206765  mitogen-activated protein kinase kinase kinase 15 [KO:K04426] [EC:2.7.11.25]
100201328  dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
100201400  stress-activated protein kinase JNK-like [KO:K04440] [EC:2.7.11.24]
100203138  apoptosis regulator BAX-like [KO:K02159]
100202650  apoptosis regulator BAX-like [KO:K02159]
100199542  apoptosis regulator BAX [KO:K02159]
100203439  apoptosis regulator BAX [KO:K02159]
100208613  bcl-2 homologous antagonist/killer-like [KO:K14021]
100212122  uncharacterized protein LOC100212122 [KO:K14021]
100203131  E3 ubiquitin-protein ligase MARCHF6 [KO:K10661] [EC:2.3.2.27]
100204107  ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
100208424  ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
100211869  ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
100210376  ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
101241018  E3 ubiquitin-protein ligase synoviolin B [KO:K10601] [EC:2.3.2.27]
100203035  E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
100210861  selenoprotein S [KO:K14025]
100214361  protein sel-1 homolog 1 [KO:K14026]
100206548  E3 ubiquitin-protein ligase AMFR-like [KO:K10636] [EC:2.3.2.36]
100210298  E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
100207010  ubiquitin-conjugating enzyme E2-17 kDa [KO:K06689] [EC:2.3.2.23]
124807926  LOW QUALITY PROTEIN: ubiquitin-conjugating enzyme E2-17 kDa-like [KO:K06689] [EC:2.3.2.23]
101237967  E3 ubiquitin-protein ligase parkin-like isoform X1 [KO:K04556] [EC:2.3.2.31]
100204043  RING-box protein 1 [KO:K03868] [EC:2.3.2.32]
100206949  cullin-1 [KO:K03347]
100212686  S-phase kinase-associated protein 1 [KO:K03094]
124809314  S-phase kinase-associated protein 1 [KO:K03094]
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
hmg00510  N-Glycan biosynthesis
hmg03050  Proteasome
hmg03060  Protein export
KO pathway
ko04141   
LinkDB

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