KEGG   PATHWAY: pprl04141
Entry
pprl04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Poeciliopsis prolifica (blackstripe livebearer)
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
pprl04141  Protein processing in endoplasmic reticulum
pprl04141

Other DBs
GO: 0030433 0034976
Organism
Poeciliopsis prolifica (blackstripe livebearer) [GN:pprl]
Gene
129351236  [KO:K09540]
129351596  man1b1b; mannosidase, alpha, class 1B, member 1b isoform X1 [KO:K23741]
129351631  [KO:K03173] [EC:2.3.2.27]
129351636  [KO:K09521]
129352487  [KO:K02159]
129352526  [KO:K08288]
129352556  [KO:K02159]
129352805  ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
129352810  ssr2; translocon-associated protein subunit beta [KO:K13250]
129353156  ubxn4; UBX domain-containing protein 4 [KO:K24348]
129353226  zgc:153993; apoptosis regulator BAX [KO:K02159]
129353242  pdia8; protein disulfide isomerase family A, member 8 isoform X1 [KO:K08056] [EC:5.3.4.1]
129353737  ern2; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
129353769  preb; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
129353874  sil1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
129353998  hspa8; heat shock cognate 71 kDa protein [KO:K03283]
129354011  hyou1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
129354086  [KO:K10950] [EC:1.8.4.-]
129354149  rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
129354179  rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 isoform X1 [KO:K12666]
129354247  [KO:K13989]
129354351  [KO:K06689] [EC:2.3.2.23]
129354417  [KO:K13249]
129354437  [KO:K10085]
129355015  [KO:K13989]
129355225  [KO:K13249]
129355297  [KO:K14007]
129355312  [KO:K03094]
129356041  [KO:K03853] [EC:3.4.22.53]
129356086  [KO:K04556] [EC:2.3.2.31]
129356397  [KO:K01367] [EC:3.4.22.52]
129356466  ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
129356716  [KO:K09525]
129356755  [KO:K14009]
129356925  [KO:K09521]
129357048  [KO:K14009]
129357727  os9; protein OS-9 isoform X1 [KO:K10088]
129357980  [KO:K28202]
129358109  [KO:K13249]
129358145  fbxo2; F-box only protein 2 isoform X1 [KO:K10099]
129358287  hsp90b1; endoplasmin [KO:K09487]
129358392  edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
129358410  [KO:K10956]
129358441  rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
129358451  [KO:K10956]
129358476  ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
129359029  ssr3; translocon-associated protein subunit gamma [KO:K13251]
129359501  bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
129359549  mapk8a; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
129359619  [KO:K01367] [EC:3.4.22.52]
129359660  capn8; calpain-8 isoform X1 [KO:K03853] [EC:3.4.22.53]
129359661  [KO:K03853] [EC:3.4.22.53]
129359829  rrbp1a; ribosome-binding protein 1a isoform X1 [KO:K14000]
129359876  dnajb12a; dnaJ homolog subfamily B member 12a [KO:K09518]
129359884  sar1ab; secretion associated, Ras related GTPase 1Ab [KO:K07953] [EC:3.6.5.-]
129359915  erlec1; endoplasmic reticulum lectin 1 [KO:K14008]
129359930  herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
129359966  [KO:K06689] [EC:2.3.2.23]
129360459  [KO:K14007]
129360462  [KO:K14007]
129360566  [KO:K08054]
129360617  [KO:K06689] [EC:2.3.2.23]
129360742  [KO:K10082]
129360743  [KO:K10082]
129360744  pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
129360883  dnajb2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
129360918  uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
129360919  dnajc3a; dnaJ homolog subfamily C member 3a isoform X1 [KO:K09523]
129361073  man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
129361384  nfe2l2a; nuclear factor erythroid 2-related factor 2a [KO:K05638]
129361729  bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
129361756  [KO:K10661] [EC:2.3.2.27]
129361940  bcl2b; apoptosis regulator Bcl-2 [KO:K02161]
129362067  dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
129362518  mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
129362599  syvn1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
129362642  ganabb; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
129362669  ube2d2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
129362811  wfs1b; wolframin isoform X1 [KO:K14020]
129362975  ubxn1; UBX domain-containing protein 1 [KO:K24348]
129363171  dnajb1a; dnaJ homolog subfamily B member 1a [KO:K09507]
129363292  hspbp1; hsp70-binding protein 1 [KO:K09562]
129363548  prkcsh; LOW QUALITY PROTEIN: glucosidase 2 subunit beta [KO:K08288]
129363690  mbtps1; LOW QUALITY PROTEIN: membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
129363741  [KO:K08852] [EC:2.7.11.1 3.1.26.-]
129363787  rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
129363883  atf4b; activating transcription factor 4b [KO:K04374]
129363905  rad23aa; RAD23 homolog A, nucleotide excision repair protein a [KO:K10839]
129364127  [KO:K10950] [EC:1.8.4.-]
129364308  [KO:K10636] [EC:2.3.2.36]
129364568  tram1; translocating chain-associated membrane protein 1 [KO:K14010]
129364593  pdia4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
129364628  dnaja2b; dnaJ homolog subfamily A member 2b [KO:K09503]
129364642  txndc5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
129364761  [KO:K10661] [EC:2.3.2.27]
129364835  sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
129365251  dnajc5gb; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma b isoform X1 [KO:K09525]
129365353  uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
129365679  si:dkey-13n15.2; protein transport protein Sec24C isoform X1 [KO:K14007]
129365891  [KO:K13989]
129366004  ufd1l; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
129366161  [KO:K04523]
129366202  xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
129366359  si:ch211-282j22.3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
129366457  traf2b; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
129366472  rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
129366538  hspa5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
129366577  man1b1a; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
129366615  rnf185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
129366819  atf4a; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374]
129366924  dnajb1b; LOW QUALITY PROTEIN: dnaJ homolog subfamily B member 1b [KO:K09507]
129367015  tmem258; transmembrane protein 258 [KO:K26502]
129367171  sec23b; protein transport protein Sec23B [KO:K14006]
129367199  zgc:136908; transitional endoplasmic reticulum ATPase [KO:K13525]
129367216  p4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
129367233  mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
129367256  tusc3; tumor suppressor candidate 3 [KO:K19478]
129367293  dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502]
129367480  derl1; derlin-1 [KO:K11519]
129367501  [KO:K07151] [EC:2.4.99.18]
129367834  sar1aa; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
129368079  sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
129368307  nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
129368329  dnajb12b; dnaJ homolog subfamily B member 12b [KO:K09518]
129368332  sec31b; protein transport protein Sec31A isoform X1 [KO:K14005]
129368376  [KO:K09542]
129368532  ube2g1a; ubiquitin-conjugating enzyme E2 G1a [KO:K10575] [EC:2.3.2.23]
129368590  stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
129368951  sel1l; protein sel-1 homolog 1 [KO:K14026]
129369092  hsp90aa1.2; heat shock protein 90, alpha (cytosolic), class A member 1, tandem duplicate 2 [KO:K04079]
129369093  hsp90aa1.1; heat shock protein HSP 90-alpha 1 [KO:K04079]
129369115  ube2j1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
129369228  sec63; LOW QUALITY PROTEIN: translocation protein SEC63 homolog [KO:K09540]
129369279  ubxn2a; UBX domain-containing protein 2A [KO:K24349]
129369808  hspa8b; heat shock protein family A (Hsp70) member 8b [KO:K03283]
129370152  dnajc5aa; dnaJ (Hsp40) homolog, subfamily C, member 5aa [KO:K09525]
129370334  zgc:113278; translocating chain-associated membrane protein 1-like 1-like [KO:K14010]
129370909  [KO:K01230] [EC:3.2.1.113]
129370974  si:ch211-1i11.3; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
129371289  krtcap2; keratinocyte-associated protein 2 [KO:K28202]
129371312  sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
129371389  [KO:K03283]
129371408  hspb6; heat shock protein beta-6 [KO:K09542]
129371777  ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
129371845  nsfl1c; NSFL1 cofactor p47 isoform X1 [KO:K14012]
129372051  vcp; transitional endoplasmic reticulum ATPase [KO:K13525]
129372101  ube2d4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
129372274  hspa1b; heat shock 70 kDa protein-like [KO:K03283]
129372319  ube4b; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
129372398  svip; small VCP/p97-interacting protein [KO:K14014]
129372550  ssr4; translocon-associated protein subunit delta [KO:K04571]
129372887  yod1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
129372890  sec13; protein SEC13 homolog [KO:K14004]
129373129  [KO:K10575] [EC:2.3.2.23]
129373277  ostc; oligosaccharyltransferase complex subunit ostc [KO:K26501]
129373298  sec24d; protein transport protein Sec24D [KO:K14007]
129373336  [KO:K08288]
129373338  plaa; LOW QUALITY PROTEIN: phospholipase A-2-activating protein [KO:K14018]
129373423  [KO:K07953] [EC:3.6.5.-]
129373425  skp1; LOW QUALITY PROTEIN: S-phase kinase-associated protein 1 [KO:K03094]
129373825  [KO:K03283]
129373828  [KO:K03283]
129374084  mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
129374086  lman1; protein ERGIC-53 [KO:K10080]
129374133  sec31a; LOW QUALITY PROTEIN: protein transport protein Sec31A [KO:K14005]
129374230  atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
129374289  [KO:K03173] [EC:2.3.2.27]
129374542  ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
129374721  atxn3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
129374841  hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
129374849  [KO:K01367] [EC:3.4.22.52]
129374990  [KO:K07151] [EC:2.4.99.18]
129375067  ubqln4; ubiquilin-4 isoform X1 [KO:K04523]
129375228  eif2s1b; eukaryotic translation initiation factor 2 subunit 1b [KO:K03237]
129375273  man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
129375300  map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
129375317  hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
129375354  dnajc5ga; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma a isoform X1 [KO:K09525]
129375358  pdia6; protein disulfide-isomerase A6 isoform X1 [KO:K09584] [EC:5.3.4.1]
129375622  ckap4; cytoskeleton-associated protein 4 [KO:K13999]
129375700  atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
129376606  edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
129377140  [KO:K09525]
129377268  [KO:K06689] [EC:2.3.2.23]
129377287  eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
129377435  sec23a; protein transport protein Sec23A [KO:K14006]
129377482  [KO:K04440] [EC:2.7.11.24]
129377517  calr; calreticulin [KO:K08057]
129377844  stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
129377885  ubxn6; UBX domain-containing protein 6 [KO:K14011]
129378041  sec62; translocation protein SEC62 [KO:K12275]
129378084  calr3b; calreticulin 3b [KO:K08057]
129378788  cryaa; alpha-crystallin A chain [KO:K09541]
129378888  mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
129379123  dnajc10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
129379302  dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
129379322  selenos; selenoprotein S isoform X1 [KO:K14025]
129379353  dnaja2a; dnaJ homolog subfamily A member 2a [KO:K09503]
129379432  capn1; calpain-1 catalytic subunit isoform X1 [KO:K01367] [EC:3.4.22.52]
129379503  dnajc3b; dnaJ homolog subfamily C member 3b [KO:K09523]
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
pprl00510  N-Glycan biosynthesis
pprl03050  Proteasome
pprl03060  Protein export
pprl04210  Apoptosis
KO pathway
ko04141   

DBGET integrated database retrieval system