KEGG   PATHWAY: dme04141
Entry
dme04141                    Pathway                                

Name
Protein processing in endoplasmic reticulum - Drosophila melanogaster (fruit fly)
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
dme04141  Protein processing in endoplasmic reticulum
dme04141

Other DBs
GO: 0030433 0034976
Organism
Drosophila melanogaster (fruit fly) [GN:dme]
Gene
Dmel_CG9539  Sec61alpha; Sec61 alpha subunit, isoform B [KO:K10956]
Dmel_CG10130  Sec61beta; Sec61 beta subunit [KO:K09481]
Dmel_CG8860  CG8860; uncharacterized protein [KO:K07342]
Dmel_CG14214  Sec61gamma; Sec61 gamma subunit, isoform B [KO:K07342]
Dmel_CG13426  CG13426; uncharacterized protein [KO:K07342]
Dmel_CG4758  Trp1; translocation protein 1, isoform C [KO:K12275]
Dmel_CG8583  Sec63; secretory 63 [KO:K09540]
Dmel_CG33303  CG33303; uncharacterized protein, isoform B [KO:K12666]
Dmel_CG6370  OstDelta; oligosaccharide transferase delta subunit, isoform B [KO:K12667]
Dmel_CG13393  Dad1; defender against apoptotic cell death 1 [KO:K12668]
Dmel_CG7830  Ostgamma; oligosaccharide transferase gamma subunit [KO:K12669]
Dmel_CG9022  Ost48; oligosaccharyltransferase 48kD subunit [KO:K12670]
Dmel_CG7748  Stt3B; catalytic subunit 3B of the oligosaccharyltransferase complex [KO:K07151] [EC:2.4.99.18]
Dmel_CG1518  Stt3A; catalytic subunit 3A of the oligosaccharyltransferase complex, isoform C [KO:K07151] [EC:2.4.99.18]
Dmel_CG1597  GCS1; glucosidase 1, isoform C [KO:K01228] [EC:3.2.1.106]
Dmel_CG10420  Sil1; Sil1 nucleotide exchange factor [KO:K14001]
Dmel_CG2918  CG2918; uncharacterized protein, isoform B [KO:K09486]
Dmel_CG4147  Hsc70-3; heat shock 70-kDa protein cognate 3, isoform E [KO:K09490] [EC:3.6.4.10]
Dmel_CG4164  shv; shriveled [KO:K09517]
Dmel_CG7556  CG7556; uncharacterized protein, isoform B [KO:K09521]
Dmel_CG8286  P58IPK; P58IPK [KO:K09523]
Dmel_CG5520  Gp93; glycoprotein 93 [KO:K09487]
Dmel_CG14476  GCS2alpha; glucosidase 2 alpha subunit, isoform B [KO:K05546] [EC:3.2.1.207]
Dmel_CG6453  GCS2beta; glucosidase 2 beta subunit, isoform B [KO:K08288]
Dmel_CG11958  Cnx99A; calnexin 99A, isoform E [KO:K08054]
Dmel_CG9906  Cnx14D; calnexin 14D [KO:K08054]
Dmel_CG1924  CG1924; uncharacterized protein [KO:K08054]
Dmel_CG8983  ERp60; endoplasmic reticulum p60 [KO:K08056] [EC:5.3.4.1]
Dmel_CG9429  Calr; calreticulin, isoform B [KO:K08057]
Dmel_CG31202  alpha-Man-Ic; alpha-Mannosidase class I c [KO:K01230] [EC:3.2.1.113]
Dmel_CG42275  alpha-Man-Ia; alpha-Mannosidase class I a, isoform J [KO:K01230] [EC:3.2.1.113]
Dmel_CG11874  alpha-Man-Ib; alpha-Mannosidase class I b [KO:K23741] [EC:3.2.1.209]
Dmel_CG5510  CG5510; uncharacterized protein [KO:K10082]
Dmel_CG6822  ergic53; ergic53, isoform A [KO:K10080]
Dmel_CG9175  CG9175; uncharacterized protein, isoform A [KO:K14003]
Dmel_CG7073  Sar1; Secretion-associated Ras-related 1, isoform A [KO:K07953] [EC:3.6.5.-]
Dmel_CG6773  Sec13; secretory 13, isoform B [KO:K14004]
Dmel_CG8266  Sec31; secretory 31, isoform C [KO:K14005]
Dmel_CG1250  Sec23; secretory 23, isoform E [KO:K14006]
Dmel_CG1472  Sec24AB; secretory 24AB, isoform B [KO:K14007]
Dmel_CG10882  Sec24CD; secretory 24CD, isoform B [KO:K14007]
Dmel_CG6850  Uggt; UDP-glucose-glycoprotein glucosyltransferase [KO:K11718] [EC:2.4.1.-]
Dmel_CG3810  Edem1; ER degradation enhancer, mannosidase alpha-like 1, isoform D [KO:K10085]
Dmel_CG5682  Edem2; ER degradation enhancer, mannosidase alpha-like 2, isoform B [KO:K10086]
Dmel_CG6988  Pdi; protein disulfide isomerase, isoform E [KO:K09580] [EC:5.3.4.1]
Dmel_CG5809  CaBP1; calcium-binding protein 1, isoform B [KO:K09584] [EC:5.3.4.1]
Dmel_CG7225  wbl; windbeutel, isoform B [KO:K09586]
Dmel_CG1837  prtp; pretaporter, isoform B [KO:K13984]
Dmel_CG18132  CG18132; uncharacterized protein [KO:K13984]
Dmel_CG1333  Ero1L; endoplasmic reticulum oxidoreductin-1-like [KO:K10950] [EC:1.8.4.-]
Dmel_CG6766  CG6766; uncharacterized protein [KO:K14008]
Dmel_CG32701  l(1)G0320; lethal (1) G0320, isoform B [KO:K13249]
Dmel_CG5474  SsRbeta; signal sequence receptor beta [KO:K13250]
Dmel_CG5885  CG5885; uncharacterized protein, isoform B [KO:K13251]
Dmel_CG9035  Tapdelta; Translocon-associated protein delta, isoform B [KO:K04571]
Dmel_CG13887  CG13887; uncharacterized protein, isoform D [KO:K14009]
Dmel_CG11642  TRAM; TRAM, isoform D [KO:K14010]
Dmel_CG10908  Der-1; Derlin-1, isoform B [KO:K11519]
Dmel_CG14899  Der-2; Derlin-2 [KO:K13989]
Dmel_CG5469  Gint3; GDI interacting protein 3, isoform D [KO:K14011]
Dmel_CG11139  p47; p47 [KO:K14012]
Dmel_CG42383  CG42383; uncharacterized protein [KO:K14012]
Dmel_CG32039  CG32039; uncharacterized protein, isoform B [KO:K14014]
Dmel_CG2331  TER94; TER94, isoform A [KO:K13525]
Dmel_CG4673  Npl4; nuclear protein localization 4, isoform A [KO:K14015]
Dmel_CG6233  Ufd1-like; ubiquitin fusion-degradation 1-like [KO:K14016]
Dmel_CG4264  Hsc70-4; heat shock protein cognate 4, isoform G [KO:K03283]
Dmel_CG18743  Hsp70Ab; Heat-shock-protein-70Ab [KO:K03283]
Dmel_CG31366  Hsp70Aa; Heat-shock-protein-70Aa [KO:K03283]
Dmel_CG7756  Hsc70-2; heat shock protein cognate 2 [KO:K03283]
Dmel_CG5436  Hsp68; heat shock protein 68 [KO:K03283]
Dmel_CG31359  Hsp70Bb; Heat-shock-protein-70Bb [KO:K03283]
Dmel_CG5834  Hsp70Bbb [KO:K03283]
Dmel_CG31449  Hsp70Ba; Heat-shock-protein-70Ba [KO:K03283]
Dmel_CG6489  Hsp70Bc; Heat-shock-protein-70Bc [KO:K03283]
Dmel_CG8937  Hsc70-1; heat shock protein cognate 1, isoform A [KO:K03283]
Dmel_CG8863  Droj2; DnaJ-like-2, isoform A [KO:K09502]
Dmel_CG9828  DnaJ-H; DnaJ homolog, isoform C [KO:K09503]
Dmel_CG3061  CG3061; uncharacterized protein [KO:K09518]
Dmel_CG30156  CG30156; uncharacterized protein, isoform B [KO:K09518]
Dmel_CG6395  Csp; cysteine string protein, isoform F [KO:K09525]
Dmel_CG1242  Hsp83; heat shock protein 83, isoform B [KO:K04079]
Dmel_CG6603  Hsc70Cb; Hsc70Cb, isoform E [KO:K09485]
Dmel_CG7945  CG7945; uncharacterized protein, isoform B [KO:K09556]
Dmel_CG10973  CG10973; uncharacterized protein [KO:K09562]
Dmel_CG4533  l(2)efl; lethal (2) essential for life, isoform C [KO:K09542]
Dmel_CG4603  CG4603; uncharacterized protein, isoform C [KO:K13719] [EC:3.1.2.-]
Dmel_CG5105  Plap; phospholipase A2 activator protein, isoform B [KO:K14018]
Dmel_CG1836  Rad23; Rad23, isoform C [KO:K10839]
Dmel_CG10694  CG10694; uncharacterized protein [KO:K10839]
Dmel_CG14224  Ubqn; ubiquilin, isoform B [KO:K04523]
Dmel_CG31528  CG31528; uncharacterized protein [KO:K04523]
Dmel_CG7865  Pngl; PNGase-like, isoform A [KO:K01456] [EC:3.5.1.52]
Dmel_CG9934  CG9934; uncharacterized protein, isoform B [KO:K10597] [EC:2.3.2.27]
Dmel_CG2087  PEK; pancreatic eIF-2alpha kinase, isoform A [KO:K08860] [EC:2.7.11.1]
Dmel_CG1609  Gcn2; Gcn2, isoform B [KO:K16196] [EC:2.7.11.1]
Dmel_CG9946  eIF2alpha; eukaryotic translation initiation factor 2 subunit alpha, isoform B [KO:K03237]
Dmel_CG8669  crc; cryptocephal, isoform E [KO:K04374]
Dmel_CG3136  Atf6; Atf6, isoform B [KO:K09054]
Dmel_CG4917  wfs1; wolfram syndrome 1, isoform A [KO:K14020]
Dmel_CG7169  S1P; S1P [KO:K08653] [EC:3.4.21.112]
Dmel_CG8988  S2P; site-2 protease [KO:K07765] [EC:3.4.24.85]
Dmel_CG9415  Xbp1; X box binding protein-1, isoform D [KO:K09027]
Dmel_CG4583  Ire1; Inositol-requiring enzyme-1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
Dmel_CG4720  Ask1; apoptotic signal-regulating kinase 1, isoform C [KO:K04426] [EC:2.7.11.25]
Dmel_CG4353  hep; hemipterous, isoform A [KO:K04431] [EC:2.7.12.2]
Dmel_CG5680  bsk; basket, isoform F [KO:K04440] [EC:2.7.11.24]
Dmel_CG1317  CG1317; uncharacterized protein, isoform C [KO:K10661] [EC:2.3.2.27]
Dmel_CG5823  CG5823; uncharacterized protein, isoform B [KO:K04554] [EC:2.3.2.23]
Dmel_CG40045  CG40045; uncharacterized protein, isoform A [KO:K10575] [EC:2.3.2.23]
Dmel_CG9602  Ubc87F; ubiquitin conjugating enzyme 87F [KO:K10575] [EC:2.3.2.23]
Dmel_CG4443  Ubc7; ubiquitin conjugating enzyme 7, isoform B [KO:K04555] [EC:2.3.2.23]
Dmel_CG1937  sip3; septin interacting protein 3, isoform B [KO:K10601] [EC:2.3.2.27]
Dmel_CG8974  CG8974; uncharacterized protein, isoform C [KO:K10666] [EC:2.3.2.27]
Dmel_CG32581  CG32581; uncharacterized protein, isoform B [KO:K10666] [EC:2.3.2.27]
Dmel_CG32847  CG32847; uncharacterized protein [KO:K10666] [EC:2.3.2.27]
Dmel_CG8141  CG8141; uncharacterized protein [KO:K10666] [EC:2.3.2.27]
Dmel_CG10221  Hrd3; HMG-coA reductase degradation 3, isoform B [KO:K14026]
Dmel_CG5203  STUB1; STIP1 homology and U-box containing protein 1 [KO:K09561] [EC:2.3.2.27]
Dmel_CG7425  eff; effete, isoform B [KO:K06689] [EC:2.3.2.23]
Dmel_CG10523  park; parkin, isoform B [KO:K04556] [EC:2.3.2.31]
Dmel_CG16982  Roc1a; regulator of cullins 1a, isoform D [KO:K03868] [EC:2.3.2.32]
Dmel_CG16988  Roc1b; regulator of cullins 1b [KO:K03868] [EC:2.3.2.32]
Dmel_CG1877  Cul1; cullin 1, isoform D [KO:K03347]
Dmel_CG11261  Cul6; cullin 6 [KO:K03347]
Dmel_CG16983  SkpA; SKP1-related A, isoform H [KO:K03094]
Dmel_CG12227  SkpF; SKP1-related F [KO:K03094]
Dmel_CG8881  SkpB; SKP1-related B [KO:K03094]
Dmel_CG11942  SkpE; SKP1-related E [KO:K03094]
Dmel_CG11941  SkpC; SKP1-related C [KO:K03094]
Dmel_CG12700  SkpD; SKP1-related D [KO:K03094]
Dmel_CG15800  CG15800; uncharacterized protein [KO:K03094]
Dmel_CG43089  CG43089; uncharacterized protein, isoform B [KO:K03094]
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
dme00510  N-Glycan biosynthesis
dme03050  Proteasome
dme03060  Protein export
KO pathway
ko04141   

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