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
129358410  protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
129358451  protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
129371312  sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
129364835  sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
129378041  sec62; translocation protein SEC62 [KO:K12275]
129351236  LOW QUALITY PROTEIN: translocation protein SEC63 homolog [KO:K09540]
129369228  sec63; LOW QUALITY PROTEIN: translocation protein SEC63 homolog [KO:K09540]
129354179  rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 isoform X1 [KO:K12666]
129358441  rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
129362067  dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
129371777  ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
129367501  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B-like [KO:K07151] [EC:2.4.99.18]
129368590  stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
129374990  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
129367256  tusc3; tumor suppressor candidate 3 [KO:K19478]
129373277  ostc; oligosaccharyltransferase complex subunit ostc [KO:K26501]
129367015  tmem258; transmembrane protein 258 [KO:K26502]
129367233  mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
129375622  ckap4; cytoskeleton-associated protein 4 [KO:K13999]
129359829  rrbp1a; ribosome-binding protein 1a isoform X1 [KO:K14000]
129353874  sil1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
129354011  hyou1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
129366538  hspa5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
129379302  dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
129356925  LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 1-like [KO:K09521]
129351636  LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 1-like [KO:K09521]
129360919  dnajc3a; dnaJ homolog subfamily C member 3a isoform X1 [KO:K09523]
129379503  dnajc3b; dnaJ homolog subfamily C member 3b [KO:K09523]
129379123  dnajc10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
129358287  hsp90b1; endoplasmin [KO:K09487]
129362642  ganabb; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
129373336  glucosidase 2 subunit beta-like [KO:K08288]
129363548  prkcsh; LOW QUALITY PROTEIN: glucosidase 2 subunit beta [KO:K08288]
129352526  glucosidase 2 subunit beta-like [KO:K08288]
129360566  calnexin-like isoform X1 [KO:K08054]
129360744  pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
129353242  pdia8; protein disulfide isomerase family A, member 8 isoform X1 [KO:K08056] [EC:5.3.4.1]
129378084  calr3b; calreticulin 3b [KO:K08057]
129377517  calr; calreticulin [KO:K08057]
129361073  man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
129375273  man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
129370909  mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
129366577  man1b1a; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
129351596  man1b1b; mannosidase, alpha, class 1B, member 1b isoform X1 [KO:K23741] [EC:3.2.1.209]
129360742  vesicular integral-membrane protein VIP36-like isoform X1 [KO:K10082]
129360743  vesicular integral-membrane protein VIP36-like isoform X1 [KO:K10082]
129374086  lman1; protein ERGIC-53 [KO:K10080]
129353769  preb; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
129373423  GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
129367834  sar1aa; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
129359884  sar1ab; secretion associated, Ras related GTPase 1Ab [KO:K07953] [EC:3.6.5.-]
129372890  sec13; protein SEC13 homolog [KO:K14004]
129368332  sec31b; protein transport protein Sec31A isoform X1 [KO:K14005]
129374133  sec31a; LOW QUALITY PROTEIN: protein transport protein Sec31A [KO:K14005]
129367171  sec23b; protein transport protein Sec23B [KO:K14006]
129377435  sec23a; protein transport protein Sec23A [KO:K14006]
129355297  protein transport protein Sec24A-like isoform X1 [KO:K14007]
129360459  protein transport protein Sec24B-like isoform X1 [KO:K14007]
129360462  protein transport protein Sec24B-like isoform X1 [KO:K14007]
129373298  sec24d; protein transport protein Sec24D [KO:K14007]
129368079  sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
129365679  si:dkey-13n15.2; protein transport protein Sec24C isoform X1 [KO:K14007]
129360918  uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
129365353  uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
129358392  edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
129354437  LOW QUALITY PROTEIN: ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
129366359  si:ch211-282j22.3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
129376606  edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
129367216  p4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
129364593  pdia4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
129375358  pdia6; protein disulfide-isomerase A6 isoform X1 [KO:K09584] [EC:5.3.4.1]
129364642  txndc5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
129364127  ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
129354086  LOW QUALITY PROTEIN: ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
129357727  os9; protein OS-9 isoform X1 [KO:K10088]
129359915  erlec1; endoplasmic reticulum lectin 1 [KO:K14008]
129355225  LOW QUALITY PROTEIN: translocon-associated protein subunit alpha-like [KO:K13249]
129358109  translocon-associated protein subunit alpha-like [KO:K13249]
129354417  translocon-associated protein subunit alpha-like [KO:K13249]
129352810  ssr2; translocon-associated protein subunit beta [KO:K13250]
129359029  ssr3; translocon-associated protein subunit gamma [KO:K13251]
129372550  ssr4; translocon-associated protein subunit delta [KO:K04571]
129356755  B-cell receptor-associated protein 31-like [KO:K14009]
129357048  LOW QUALITY PROTEIN: B-cell receptor-associated protein 31-like [KO:K14009]
129364568  tram1; translocating chain-associated membrane protein 1 [KO:K14010]
129370334  zgc:113278; translocating chain-associated membrane protein 1-like 1-like [KO:K14010]
129367480  derl1; derlin-1 [KO:K11519]
129355015  LOW QUALITY PROTEIN: derlin-2-like [KO:K13989]
129365891  derlin-2-like [KO:K13989]
129354247  LOW QUALITY PROTEIN: derlin-2-like [KO:K13989]
129362975  ubxn1; UBX domain-containing protein 1 [KO:K24348]
129353156  ubxn4; UBX domain-containing protein 4 [KO:K24348]
129369279  ubxn2a; UBX domain-containing protein 2A [KO:K24349]
129377885  ubxn6; UBX domain-containing protein 6 [KO:K14011]
129371845  nsfl1c; NSFL1 cofactor p47 isoform X1 [KO:K14012]
129372398  svip; small VCP/p97-interacting protein [KO:K14014]
129372051  vcp; transitional endoplasmic reticulum ATPase [KO:K13525]
129367199  zgc:136908; transitional endoplasmic reticulum ATPase [KO:K13525]
129368307  nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
129366004  ufd1l; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
129372274  hspa1b; heat shock 70 kDa protein-like [KO:K03283]
129373825  LOW QUALITY PROTEIN: heat shock 70 kDa protein 1-like [KO:K03283]
129373828  LOW QUALITY PROTEIN: heat shock 70 kDa protein 1-like [KO:K03283]
129369808  hspa8b; heat shock protein family A (Hsp70) member 8b [KO:K03283]
129353998  hspa8; heat shock cognate 71 kDa protein [KO:K03283]
129371389  LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein-like [KO:K03283]
129367293  dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502]
129379353  dnaja2a; dnaJ homolog subfamily A member 2a [KO:K09503]
129364628  dnaja2b; dnaJ homolog subfamily A member 2b [KO:K09503]
129366924  dnajb1b; LOW QUALITY PROTEIN: dnaJ homolog subfamily B member 1b [KO:K09507]
129363171  dnajb1a; dnaJ homolog subfamily B member 1a [KO:K09507]
129360883  dnajb2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
129368329  dnajb12b; dnaJ homolog subfamily B member 12b [KO:K09518]
129359876  dnajb12a; dnaJ homolog subfamily B member 12a [KO:K09518]
129356716  dnaJ homolog subfamily C member 5-like [KO:K09525]
129375354  dnajc5ga; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma a isoform X1 [KO:K09525]
129370152  dnajc5aa; dnaJ (Hsp40) homolog, subfamily C, member 5aa [KO:K09525]
129365251  dnajc5gb; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma b isoform X1 [KO:K09525]
129377140  dnaJ homolog subfamily C member 5-like [KO:K09525]
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]
129375317  hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
129374841  hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
129361729  bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
129359501  bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
129363292  hspbp1; hsp70-binding protein 1 [KO:K09562]
129378788  cryaa; alpha-crystallin A chain [KO:K09541]
129368376  alpha-crystallin B chain-like [KO:K09542]
129371408  hspb6; heat shock protein beta-6 [KO:K09542]
129372887  yod1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
129373338  plaa; LOW QUALITY PROTEIN: phospholipase A-2-activating protein [KO:K14018]
129366472  rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
129363905  rad23aa; RAD23 homolog A, nucleotide excision repair protein a [KO:K10839]
129366161  ubiquilin-1-like [KO:K04523]
129375067  ubqln4; ubiquilin-4 isoform X1 [KO:K04523]
129352805  ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
129374721  atxn3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
129372319  ube4b; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
129377287  eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
129375228  eif2s1b; eukaryotic translation initiation factor 2 subunit 1b [KO:K03237]
129361384  nfe2l2a; nuclear factor erythroid 2-related factor 2a [KO:K05638]
129366819  atf4a; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374]
129363883  atf4b; activating transcription factor 4b [KO:K04374]
129358476  ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
129361940  bcl2b; apoptosis regulator Bcl-2 [KO:K02161]
129374230  atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
129375700  atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
129362811  wfs1b; wolframin isoform X1 [KO:K14020]
129363690  mbtps1; LOW QUALITY PROTEIN: membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
129378888  mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
129366202  xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
129363741  serine/threonine-protein kinase/endoribonuclease IRE1-like [KO:K08852] [EC:2.7.11.1 3.1.26.-]
129353737  ern2; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
129366457  traf2b; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
129374289  TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
129351631  TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
129375300  map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
129370974  si:ch211-1i11.3; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
129362518  mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
129374084  mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
129359549  mapk8a; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
129377482  mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
129352487  apoptosis regulator BAX-like isoform X1 [KO:K02159]
129352556  apoptosis regulator BAX-like isoform X1 [KO:K02159]
129353226  zgc:153993; apoptosis regulator BAX [KO:K02159]
129379432  capn1; calpain-1 catalytic subunit isoform X1 [KO:K01367] [EC:3.4.22.52]
129356397  LOW QUALITY PROTEIN: calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
129374849  LOW QUALITY PROTEIN: calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
129359619  calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
129356041  calpain-2 catalytic subunit-like [KO:K03853] [EC:3.4.22.53]
129359660  capn8; calpain-8 isoform X1 [KO:K03853] [EC:3.4.22.53]
129359661  calpain-2 catalytic subunit-like [KO:K03853] [EC:3.4.22.53]
129361756  E3 ubiquitin-protein ligase MARCHF6-like [KO:K10661] [EC:2.3.2.27]
129364761  E3 ubiquitin-protein ligase MARCHF6-like [KO:K10661] [EC:2.3.2.27]
129369115  ube2j1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
129356466  ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
129373129  ubiquitin-conjugating enzyme E2 G1-like [KO:K10575] [EC:2.3.2.23]
129368532  ube2g1a; ubiquitin-conjugating enzyme E2 G1a [KO:K10575] [EC:2.3.2.23]
129374542  ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
129362599  syvn1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
129366615  rnf185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
129354149  rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
129379322  selenos; selenoprotein S isoform X1 [KO:K14025]
129368951  sel1l; protein sel-1 homolog 1 [KO:K14026]
129359930  herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
129364308  E3 ubiquitin-protein ligase AMFR-like [KO:K10636] [EC:2.3.2.36]
129377844  stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
129360617  ubiquitin-conjugating enzyme E2 2-like isoform X1 [KO:K06689] [EC:2.3.2.23]
129372101  ube2d4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
129362669  ube2d2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
129359966  ubiquitin-conjugating enzyme E2 D2-like [KO:K06689] [EC:2.3.2.23]
129377268  ubiquitin-conjugating enzyme E2 D2-like isoform X1 [KO:K06689] [EC:2.3.2.23]
129354351  ubiquitin-conjugating enzyme E2 2-like [KO:K06689] [EC:2.3.2.23]
129356086  E3 ubiquitin-protein ligase parkin-like [KO:K04556] [EC:2.3.2.31]
129363787  rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
129355312  LOW QUALITY PROTEIN: S-phase kinase-associated protein 1-like [KO:K03094]
129373425  skp1; LOW QUALITY PROTEIN: S-phase kinase-associated protein 1 [KO:K03094]
129358145  fbxo2; F-box only protein 2 isoform X1 [KO:K10099]
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
pprl00510  N-Glycan biosynthesis
pprl03050  Proteasome
pprl03060  Protein export
pprl04210  Apoptosis
KO pathway
ko04141   
LinkDB

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