KEGG   PATHWAY: pxu04141
Entry
pxu04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Papilio xuthus (Asian swallowtail)
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
pxu04141  Protein processing in endoplasmic reticulum
pxu04141

Other DBs
GO: 0030433 0034976
Organism
Papilio xuthus (Asian swallowtail) [GN:pxu]
Gene
106125348  protein transport protein Sec61 subunit alpha isoform 2 [KO:K10956]
106127613  protein transport protein Sec61 subunit beta [KO:K09481]
106115520  translocation protein SEC62 isoform X1 [KO:K12275]
106125426  translocation protein SEC63 homolog [KO:K09540]
106123924  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
106124474  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
106128399  dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
106114703  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
106120261  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
106113444  tumor suppressor candidate 3 [KO:K19478]
106127498  oligosaccharyltransferase complex subunit ostc [KO:K26501]
106117243  transmembrane protein 258 [KO:K26502]
106122821  mannosyl-oligosaccharide glucosidase isoform X1 [KO:K01228] [EC:3.2.1.106]
106121685  hypoxia up-regulated protein 1 [KO:K09486]
106117184  heat shock 70 kDa protein cognate 3 [KO:K09490] [EC:3.6.4.10]
106118123  dnaJ homolog subfamily B member 11 [KO:K09517]
106127011  uncharacterized protein F54F2.9 [KO:K09521]
106127754  dnaJ homolog subfamily C member 3 [KO:K09523]
106125701  dnaJ homolog subfamily C member 10-like [KO:K09530] [EC:1.8.4.-]
106125324  endoplasmin [KO:K09487]
106125787  neutral alpha-glucosidase AB [KO:K05546] [EC:3.2.1.207]
106122574  glucosidase 2 subunit beta [KO:K08288]
106123489  calnexin isoform X1 [KO:K08054]
106124241  protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
106125395  calreticulin [KO:K08057]
106115883  mannosyl-oligosaccharide alpha-1,2-mannosidase isoform A [KO:K01230] [EC:3.2.1.113]
106126459  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741] [EC:3.2.1.209]
106116139  vesicular integral-membrane protein VIP36 [KO:K10082]
106124433  protein ERGIC-53 [KO:K10080]
106127492  LOW QUALITY PROTEIN: SEC12-like protein 1 [KO:K14003]
106115953  GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
106120317  protein SEC13 homolog [KO:K14004]
106126507  protein transport protein Sec31A [KO:K14005]
106120216  protein transport protein Sec23A isoform X1 [KO:K14006]
106123147  protein transport protein Sec24C isoform X1 [KO:K14007]
106125327  protein transport protein Sec24A [KO:K14007]
106128844  basic salivary proline-rich protein 2-like [KO:K14007]
106117253  UDP-glucose:glycoprotein glucosyltransferase isoform X1 [KO:K11718] [EC:2.4.1.-]
106123491  ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
106126229  ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
106121216  protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
106124663  protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
106117637  endoplasmic reticulum resident protein 29 [KO:K09586]
106122370  thioredoxin domain-containing protein 5-like [KO:K13984] [EC:5.3.4.1]
106128446  ero1-like protein [KO:K10950] [EC:1.8.4.-]
106125514  endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
106121002  translocon-associated protein subunit alpha [KO:K13249]
106124843  translocon-associated protein subunit beta [KO:K13250]
106115951  translocon-associated protein subunit gamma [KO:K13251]
106122767  translocon-associated protein subunit delta [KO:K04571]
106119336  B-cell receptor-associated protein 31-like [KO:K14009]
106119659  B-cell receptor-associated protein 31 [KO:K14009]
106124793  translocating chain-associated membrane protein 1 [KO:K14010]
106126719  derlin-1 [KO:K11519]
106115615  derlin-2 [KO:K13989]
106124046  UBX domain-containing protein 1 [KO:K24348]
106124674  UBX domain-containing protein 4 isoform X1 [KO:K24348]
106118634  UBX domain-containing protein 6 [KO:K14011]
106115298  NSFL1 cofactor p47 [KO:K14012]
106121926  small VCP/p97-interacting protein [KO:K14014]
106114291  transitional endoplasmic reticulum ATPase [KO:K13525]
106121369  nuclear protein localization protein 4 homolog [KO:K14015]
106117638  ubiquitin fusion degradation protein 1 homolog isoform X1 [KO:K14016]
106114408  heat shock protein 68-like [KO:K03283]
106122546  heat shock protein 68 [KO:K03283]
106115795  heat shock 70 kDa protein cognate 4 [KO:K03283]
106117831  heat shock protein 68-like [KO:K03283]
106118619  heat shock protein 68-like [KO:K03283]
106118649  heat shock protein 68-like [KO:K03283]
106118664  heat shock protein 68-like [KO:K03283]
106115940  dnaJ homolog subfamily A member 1 [KO:K09502]
106127709  dnaJ homolog subfamily A member 2-like [KO:K09503]
106124733  dnaJ protein homolog 1 [KO:K09507]
106122866  dnaJ homolog subfamily B member 14 [KO:K09518]
106117263  dnaJ homolog subfamily C member 5 [KO:K09525]
106118617  heat shock protein 83 [KO:K04079]
106123401  97 kDa heat shock protein isoform X1 [KO:K09485]
106114245  BAG family molecular chaperone regulator 2 [KO:K09556]
106117614  hsp70-binding protein 1 [KO:K09562]
106114468  protein lethal(2)essential for life [KO:K09542]
106121935  protein lethal(2)essential for life-like [KO:K09542]
106123085  protein lethal(2)essential for life-like [KO:K09542]
106123120  protein lethal(2)essential for life-like [KO:K09542]
106123167  protein lethal(2)essential for life-like [KO:K09542]
106123168  protein lethal(2)essential for life-like [KO:K09542]
106124043  protein lethal(2)essential for life-like [KO:K09542]
106124062  protein lethal(2)essential for life-like [KO:K09542]
106124586  protein lethal(2)essential for life-like [KO:K09542]
106118556  protein lethal(2)essential for life-like [KO:K09542]
106124080  heat shock protein beta-11-like [KO:K09542]
106116021  ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
106125445  phospholipase A-2-activating protein-like [KO:K14018]
106128237  UV excision repair protein RAD23 homolog A [KO:K10839]
106119494  ubiquilin-1 [KO:K04523]
106114026  peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
106126755  ubiquitin conjugation factor E4 B-like [KO:K10597] [EC:2.3.2.27]
106116788  eukaryotic translation initiation factor 2-alpha kinase-like isoform X1 [KO:K08860] [EC:2.7.11.1]
106119975  eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
106121235  activating transcription factor of chaperone isoform X1 [KO:K04374]
106117195  cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
106116441  wolframin isoform X1 [KO:K14020]
106113860  membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
106121895  membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
106128541  X-box-binding protein 1 [KO:K09027]
106119136  serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
106126710  mitogen-activated protein kinase kinase kinase 15 isoform X1 [KO:K04426] [EC:2.7.11.25]
106117320  dual specificity mitogen-activated protein kinase kinase 7-like isoform X1 [KO:K04431] [EC:2.7.12.2]
106125616  stress-activated protein kinase JNK isoform X1 [KO:K04440] [EC:2.7.11.24]
106121219  E3 ubiquitin-protein ligase MARCH6 isoform X1 [KO:K10661] [EC:2.3.2.27]
106120254  ubiquitin-conjugating enzyme E2 J1-like [KO:K10578] [EC:2.3.2.23]
106119317  ubiquitin-conjugating enzyme E2 J2-like [KO:K04554] [EC:2.3.2.23]
106117791  ubiquitin-conjugating enzyme E2 G1 isoform X1 [KO:K10575] [EC:2.3.2.23]
106126457  ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
106120245  E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
106128384  E3 ubiquitin-protein ligase RNF185-like [KO:K10666] [EC:2.3.2.27]
106115305  uncharacterized protein LOC106115305 [KO:K14025]
106119891  protein sel-1 homolog 1 [KO:K14026]
106113967  E3 ubiquitin-protein ligase hrd-like protein 1 [KO:K10636] [EC:2.3.2.36]
106118819  E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
106127217  ubiquitin-conjugating enzyme E2-17 kDa [KO:K06689] [EC:2.3.2.23]
106123473  RING-box protein 1A [KO:K03868] [EC:2.3.2.32]
106124644  cullin-1 [KO:K03347]
106123556  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
pxu00510  N-Glycan biosynthesis
pxu03050  Proteasome
pxu03060  Protein export
KO pathway
ko04141   
LinkDB

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