KEGG   PATHWAY: pswi04141
Entry
pswi04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Pseudoliparis swirei (Mariana hadal snailfish)
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
pswi04141  Protein processing in endoplasmic reticulum
pswi04141

Other DBs
GO: 0030433 0034976
Organism
Pseudoliparis swirei (Mariana hadal snailfish) [GN:pswi]
Gene
130188317  dnajb1a; dnaJ homolog subfamily B member 1a [KO:K09507]
130188416  hspbp1; hsp70-binding protein 1 [KO:K09562]
130188433  ern1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
130188618  atf4b; LOW QUALITY PROTEIN: activating transcription factor 4b [KO:K04374]
130188662  [KO:K03283]
130189005  rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
130189025  [KO:K02159]
130189141  prkcsh; glucosidase 2 subunit beta [KO:K08288]
130189226  rad23aa; RAD23 homolog A, nucleotide excision repair protein a [KO:K10839]
130189520  ern2; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
130189597  mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
130189817  dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502]
130189903  dnajb1b; dnaJ homolog subfamily B member 1b [KO:K09507]
130190034  atf4a; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
130190042  map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
130190073  tusc3; tumor suppressor candidate 3 [KO:K19478]
130190327  wfs1a; wolframin [KO:K14020]
130190352  p4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
130190650  [KO:K14006]
130190892  dnajc1; dnaJ homolog subfamily C member 1 [KO:K09521]
130190941  [KO:K09525]
130190951  [KO:K09542]
130190995  ero1b; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
130191194  ubxn4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
130191238  [KO:K03853] [EC:3.4.22.53]
130191246  [KO:K03853] [EC:3.4.22.53]
130191784  ube2g1a; ubiquitin-conjugating enzyme E2 G1a [KO:K10575] [EC:2.3.2.23]
130191917  rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
130191938  [KO:K01367] [EC:3.4.22.52]
130192048  hspa8; heat shock cognate 71 kDa protein [KO:K03283]
130192230  selenos; selenoprotein S [KO:K14025]
130192351  [KO:K03283]
130192556  dnaja2a; dnaJ homolog subfamily A member 2a [KO:K09503]
130192737  zgc:153993; apoptosis regulator BAX [KO:K02159]
130192934  [KO:K10636] [EC:2.3.2.36]
130193023  ero1a; ERO1-like protein alpha isoform X1 [KO:K10950] [EC:1.8.4.-]
130193519  [KO:K09054]
130193551  calr; calreticulin [KO:K08057]
130193663  edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
130194091  calr3a; calreticulin 3a [KO:K08057]
130194475  ubxn6; UBX domain-containing protein 6 [KO:K14011]
130194858  mbtps1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
130194999  [KO:K04523]
130195109  dnaja2b; dnaJ homolog subfamily A member 2b [KO:K09503]
130195301  tmem258; transmembrane protein 258 [KO:K26502]
130195479  hsp90b1; endoplasmin [KO:K09487]
130195639  svip; small VCP/p97-interacting protein [KO:K14014]
130195728  pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
130195772  ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
130196346  man1b1b; mannosidase, alpha, class 1B, member 1b [KO:K23741]
130196482  xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
130196697  [KO:K03173] [EC:2.3.2.27]
130197003  ufd1l; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
130197062  ube2d4; ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
130197110  ssr2; translocon-associated protein subunit beta [KO:K13250]
130197175  [KO:K10575] [EC:2.3.2.23]
130197186  derl3; derlin-3 isoform X1 [KO:K13989]
130197333  si:dkey-13n15.2; LOW QUALITY PROTEIN: protein transport protein Sec24C [KO:K14007]
130197731  sec62; translocation protein SEC62 [KO:K12275]
130198144  bcl2b; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
130198179  dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
130198442  [KO:K10661] [EC:2.3.2.27]
130198538  [KO:K08057]
130198579  [KO:K08056] [EC:5.3.4.1]
130198637  bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
130198887  nsfl1c; NSFL1 cofactor p47 [KO:K14012]
130198901  [KO:K09525]
130198936  [KO:K07151] [EC:2.4.99.18]
130199029  bcap31; B-cell receptor-associated protein 31 [KO:K14009]
130199340  [KO:K10100]
130199412  ssr4; translocon-associated protein subunit delta [KO:K04571]
130199688  dnajc5aa; dnaJ (Hsp40) homolog, subfamily C, member 5aa isoform X1 [KO:K09525]
130199916  zgc:113278; translocating chain-associated membrane protein 1-like 1-like [KO:K14010]
130199976  ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
130200082  dnajb2; dnaJ homolog subfamily B member 2 [KO:K09508]
130200125  ube4b; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
130200126  [KO:K10597] [EC:2.3.2.27]
130200189  edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
130200878  ckap4; cytoskeleton-associated protein 4 [KO:K13999]
130200965  hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
130201073  dnajc5gb; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma b [KO:K09525]
130201218  atxn3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
130201407  sec23a; protein transport protein Sec23A [KO:K14006]
130201552  eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
130201677  [KO:K06689] [EC:2.3.2.23]
130202004  uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
130202236  man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA isoform X1 [KO:K01230] [EC:3.2.1.113]
130202259  sel1l; protein sel-1 homolog 1 isoform X1 [KO:K14026]
130202447  hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
130202493  eif2s1b; eukaryotic translation initiation factor 2 subunit 1b [KO:K03237]
130202514  ubxn2a; UBX domain-containing protein 2A [KO:K24349]
130202581  pdia6; protein disulfide-isomerase A6 isoform X1 [KO:K09584] [EC:5.3.4.1]
130202610  dnajc5ga; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma a isoform X1 [KO:K09525]
130202778  sec63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
130202916  ube2j1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
130203073  hsp90aa1.2; heat shock protein 90, alpha (cytosolic), class A member 1, tandem duplicate 2 [KO:K04079]
130203074  hsp90aa1.1; heat shock protein HSP 90-alpha 1 [KO:K04079]
130203090  map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
130203285  ube2d1b; ubiquitin-conjugating enzyme E2 D1b [KO:K06689] [EC:2.3.2.23]
130203361  mapk8b; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
130203414  sec31b; protein transport protein Sec31A [KO:K14005]
130203424  nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
130203443  sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
130203571  dnajb12b; dnaJ homolog subfamily B member 12b [KO:K09518]
130203754  stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
130203872  sar1aa; GTP-binding protein SAR1b isoform X1 [KO:K07953] [EC:3.6.5.-]
130204560  uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
130204573  dnajc3a; dnaJ homolog subfamily C member 3a [KO:K09523]
130204860  traf2b; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
130204977  sec31a; LOW QUALITY PROTEIN: protein transport protein Sec31A [KO:K14005]
130205033  ube2g1b; ubiquitin-conjugating enzyme E2G 1b [KO:K10575] [EC:2.3.2.23]
130205107  rnf185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
130205143  rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
130205351  ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
130205358  mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
130205479  hspa5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
130205485  lman1; protein ERGIC-53 [KO:K10080]
130205488  man1b1a; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
130205564  si:ch211-282j22.3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
130205654  [KO:K01367] [EC:3.4.22.52]
130205745  [KO:K03173] [EC:2.3.2.27]
130205936  marchf6; E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
130205957  [KO:K03347]
130205975  txndc5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
130205982  man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
130206050  [KO:K07151] [EC:2.4.99.18]
130206054  mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
130206134  preb; prolactin regulatory element-binding protein [KO:K14003]
130206187  pdia4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
130206381  sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
130206471  tram1; translocating chain-associated membrane protein 1 [KO:K14010]
130206514  ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
130206770  dnajc10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
130206778  mapk8a; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
130206819  [KO:K06689] [EC:2.3.2.23]
130206983  [KO:K03853] [EC:3.4.22.53]
130207002  dnajb12a; dnaJ homolog subfamily B member 12a [KO:K09518]
130207167  rrbp1a; ribosome-binding protein 1a isoform X1 [KO:K14000]
130207384  bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
130207409  erlec1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
130207436  [KO:K03853] [EC:3.4.22.53]
130207520  nfe2l2a; nuclear factor erythroid 2-related factor 2a [KO:K05638]
130207599  sar1ab; secretion associated, Ras related GTPase 1Ab isoform X1 [KO:K07953] [EC:3.6.5.-]
130207628  [KO:K01367] [EC:3.4.22.52]
130207935  fbxo2; F-box only protein 2 isoform X1 [KO:K10099]
130208070  edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
130208223  rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
130208280  [KO:K13249]
130208282  ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
130208365  yod1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
130208676  [KO:K10956]
130208678  sec61a1a; protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
130208686  os9; protein OS-9 [KO:K10088]
130208872  rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
130208937  sec13; protein SEC13 homolog [KO:K14004]
130209211  ube2d2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
130209218  [KO:K19478]
130209344  syvn1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
130209354  [KO:K06689] [EC:2.3.2.23]
130209413  ganabb; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
130209416  sec24b; protein transport protein Sec24B isoform X1 [KO:K14007]
130209426  mapk9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
130209733  canx; calnexin isoform X1 [KO:K08054]
130209744  sil1; nucleotide exchange factor SIL1 [KO:K14001]
130209912  lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
130210023  ubxn1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
130210241  wfs1b; wolframin [KO:K14020]
130210285  [KO:K03283]
130210419  ssr3; translocon-associated protein subunit gamma [KO:K13251]
130210884  sec24a; protein transport protein Sec24A isoform X1 [KO:K14007]
130211289  sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
130211296  skp1; S-phase kinase-associated protein 1 [KO:K03094]
130211694  [KO:K08288]
130211794  plaa; phospholipase A-2-activating protein [KO:K14018]
130211899  ostc; oligosaccharyltransferase complex subunit ostc [KO:K26501]
130211975  dnajc3b; dnaJ homolog subfamily C member 3b [KO:K09523]
130212011  stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
130212012  sec24d; protein transport protein Sec24D [KO:K14007]
130212112  capn1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
130212557  [KO:K01230] [EC:3.2.1.113]
130212722  atf6b; LOW QUALITY PROTEIN: cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
130212748  krtcap2; keratinocyte-associated protein 2 [KO:K28202]
130212807  derl1; derlin-1 [KO:K11519]
130212815  sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
130212975  si:ch211-1i11.3; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
130213322  hsc70; heat shock cognate 71 kDa protein [KO:K03283]
130213342  hspb6; heat shock protein beta-6 [KO:K09542]
130213414  hyou1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
130213513  [KO:K04523]
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
pswi00510  N-Glycan biosynthesis
pswi03050  Proteasome
pswi03060  Protein export
pswi04210  Apoptosis
KO pathway
ko04141   

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