KEGG   PATHWAY: gaf04141
Entry
gaf04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Gambusia affinis (western mosquitofish)
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
gaf04141  Protein processing in endoplasmic reticulum
gaf04141

Other DBs
GO: 0030433 0034976
Organism
Gambusia affinis (western mosquitofish) [GN:gaf]
Gene
122834345  protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
122834687  sec61a1; protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
122831233  sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
122824537  sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
122841836  sec62; translocation protein SEC62 [KO:K12275]
122825424  sec63; translocation protein SEC63 homolog [KO:K09540]
122834374  rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
122834016  rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
122841825  dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
122824770  ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
122845175  stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
122831300  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B-like [KO:K07151] [EC:2.4.99.18]
122843380  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
122836855  magnesium transporter protein 1-like isoform X1 [KO:K19478]
122829888  tusc3; tumor suppressor candidate 3 [KO:K19478]
122820616  ostc; oligosaccharyltransferase complex subunit ostc [KO:K26501]
122835708  tmem258; transmembrane protein 258 [KO:K26502]
122829917  mogs; mannosyl-oligosaccharide glucosidase isoform X1 [KO:K01228] [EC:3.2.1.106]
122826183  ckap4; cytoskeleton-associated protein 4 [KO:K13999]
122842937  rrbp1a; ribosome-binding protein 1a isoform X1 [KO:K14000]
122837043  sil1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
122845065  hyou1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
122819399  hspa5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
122839502  dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
122841838  dnajc1; dnaJ homolog subfamily C member 1 [KO:K09521]
122820100  dnajc3b; dnaJ homolog subfamily C member 3b [KO:K09523]
122839373  dnajc3a; dnaJ homolog subfamily C member 3a isoform X1 [KO:K09523]
122840117  dnajc10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
122835227  hsp90b1; endoplasmin [KO:K09487]
122836958  ganabb; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
122820601  glucosidase 2 subunit beta-like isoform X1 [KO:K08288]
122821538  prkcsh; glucosidase 2 subunit beta [KO:K08288]
122836729  canx; calnexin isoform X1 [KO:K08054]
122843872  pdia8; protein disulfide isomerase family A, member 8 [KO:K08056] [EC:5.3.4.1]
122835858  pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
122841566  calr3b; calreticulin 3b [KO:K08057]
122838972  calr; calreticulin [KO:K08057]
122825267  man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
122831705  mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
122839778  man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
122819517  man1b1a; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
122827851  man1b1b; mannosidase, alpha, class 1B, member 1b [KO:K23741] [EC:3.2.1.209]
122836857  lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
122819609  lman1; protein ERGIC-53 [KO:K10080]
122830656  preb; prolactin regulatory element-binding protein [KO:K14003]
122845271  sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
122843011  sar1ab; secretion associated, Ras related GTPase 1Ab [KO:K07953] [EC:3.6.5.-]
122822887  sar1aa; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
122834558  sec13; protein SEC13 homolog [KO:K14004]
122823317  sec31b; protein transport protein Sec31A [KO:K14005]
122819745  sec31a; protein transport protein Sec31A isoform X1 [KO:K14005]
122846057  sec23a; protein transport protein Sec23A [KO:K14006]
122829265  sec23b; protein transport protein Sec23B [KO:K14006]
122823481  sec24c; LOW QUALITY PROTEIN: protein transport protein Sec24C [KO:K14007]
122837056  sec24b; protein transport protein Sec24B isoform X1 [KO:K14007]
122844929  sec24a; protein transport protein Sec24A isoform X1 [KO:K14007]
122819823  sec24d; protein transport protein Sec24D isoform X1 [KO:K14007]
122828220  si:dkey-13n15.2; protein transport protein Sec24C [KO:K14007]
122846263  uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
122839372  uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
122833593  edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
122837675  edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
122819511  si:ch211-282j22.3; LOW QUALITY PROTEIN: ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
122838207  edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
122829062  p4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
122823914  pdia4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
122825779  pdia6; protein disulfide-isomerase A6 isoform X1 [KO:K09584] [EC:5.3.4.1]
122824019  txndc5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
122845256  ero1a; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
122829831  ero1b; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
122834727  os9; protein OS-9 isoform X1 [KO:K10088]
122842550  erlec1; endoplasmic reticulum lectin 1 [KO:K14008]
122824453  ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
122833990  translocon-associated protein subunit alpha-like [KO:K13249]
122843966  ssr2; translocon-associated protein subunit beta [KO:K13250]
122833282  ssr3; translocon-associated protein subunit gamma [KO:K13251]
122837650  ssr4; translocon-associated protein subunit delta [KO:K04571]
122843705  bcap31; B-cell receptor-associated protein 31 [KO:K14009]
122824166  tram1; translocating chain-associated membrane protein 1 [KO:K14010]
122829336  zgc:113278; translocating chain-associated membrane protein 1-like 1-like [KO:K14010]
122831536  derl1; derlin-1 [KO:K11519]
122847214  derl2; derlin-2 [KO:K13989]
122828211  derlin-3-like isoform X1 [KO:K13989]
122836956  ubxn1; UBX domain-containing protein 1 [KO:K24348]
122840126  ubxn4; UBX domain-containing protein 4 [KO:K24348]
122825232  ubxn2a; UBX domain-containing protein 2A [KO:K24349]
122838422  ubxn6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
122830620  nsfl1c; NSFL1 cofactor p47 isoform X1 [KO:K14012]
122835393  small VCP/p97-interacting protein isoform X1 [KO:K14014]
122828727  transitional endoplasmic reticulum ATPase [KO:K13525]
122829701  zgc:136908; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
122823191  nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
122826546  ufd1l; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
122837131  hspa1b; heat shock 70 kDa protein-like [KO:K03283]
122845062  hspa8; heat shock cognate 71 kDa protein [KO:K03283]
122831349  hsc70; heat shock cognate 71 kDa protein [KO:K03283]
122832704  hspa8b; heat shock protein family A (Hsp70) member 8b [KO:K03283]
122821641  heat shock 70 kDa protein 1 [KO:K03283]
122829405  dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502]
122845463  dnaja2a; dnaJ homolog subfamily A member 2a [KO:K09503]
122835530  dnaja2b; dnaJ homolog subfamily A member 2b [KO:K09503]
122821454  dnajb1a; dnaJ homolog subfamily B member 1a [KO:K09507]
122829702  dnajb1b; dnaJ homolog subfamily B member 1b [KO:K09507]
122840343  dnajb2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
122823315  dnajb12b; dnaJ homolog subfamily B member 12b [KO:K09518]
122843018  dnajb12a; dnaJ homolog subfamily B member 12a isoform X1 [KO:K09518]
122845563  dnajc5gb; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma b isoform X1 [KO:K09525]
122841611  dnaJ homolog subfamily C member 5-like [KO:K09525]
122825207  dnajc5ga; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma a isoform X1 [KO:K09525]
122832431  dnajc5ab; dnaJ homolog subfamily C member 5 [KO:K09525]
122832796  dnajc5aa; dnaJ (Hsp40) homolog, subfamily C, member 5aa [KO:K09525]
122825483  hsp90aa1.2; heat shock protein 90, alpha (cytosolic), class A member 1, tandem duplicate 2 [KO:K04079]
122825484  hsp90aa1.1; heat shock protein HSP 90-alpha 1 [KO:K04079]
122825720  hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
122846597  hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
122841849  bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
122842472  bag2; BAG family molecular chaperone regulator 2 isoform X1 [KO:K09556]
122822355  hspbp1; hsp70-binding protein 1 [KO:K09562]
122827008  cryaa; alpha-crystallin A chain [KO:K09541]
122844426  cryabb; crystallin, alpha B, b isoform X1 [KO:K09542]
122831378  hspb6; heat shock protein beta-6 isoform X1 [KO:K09542]
122833812  yod1; ubiquitin thioesterase OTU1 isoform X1 [KO:K13719] [EC:3.1.2.-]
122820397  plaa; phospholipase A-2-activating protein isoform X1 [KO:K14018]
122819644  rad23b; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
122821405  rad23aa; RAD23 homolog A, nucleotide excision repair protein a [KO:K10839]
122828715  ubiquilin-1-like isoform X1 [KO:K04523]
122843644  ubqln4; ubiquilin-4 isoform X1 [KO:K04523]
122843185  ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
122846676  atxn3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
122834449  ube4b; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
122846077  eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
122825718  eif2s1b; eukaryotic translation initiation factor 2 subunit 1b [KO:K03237]
122840386  nfe2l2a; nuclear factor erythroid 2-related factor 2a [KO:K05638]
122828240  nfe2; transcription factor NF-E2 45 kDa subunit [KO:K05638]
122830317  atf4a; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
122822234  atf4b; activating transcription factor 4b [KO:K04374]
122833414  ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
122841204  bcl2b; apoptosis regulator Bcl-2 [KO:K02161]
122839087  atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
122844144  atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
122836643  wfs1b; wolframin [KO:K14020]
122829078  wfs1a; wolframin isoform X1 [KO:K14020]
122834955  mbtps1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
122823952  mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
122828669  xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
122822512  serine/threonine-protein kinase/endoribonuclease IRE1-like [KO:K08852] [EC:2.7.11.1 3.1.26.-]
122829844  ern2; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
122819276  TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
122819462  traf2b; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
122827742  TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
122825130  map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
122831685  si:ch211-1i11.3; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
122830227  map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
122846859  mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
122823403  mapk8b; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
122842229  mapk8a; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
122836543  mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
122819457  zgc:153993; apoptosis regulator BAX [KO:K02159]
122821472  apoptosis regulator BAX-like isoform X1 [KO:K02159]
122821473  apoptosis regulator BAX-like [KO:K02159]
122845740  calpain-A-like [KO:K01367] [EC:3.4.22.52]
122845742  calpain-A-like [KO:K01367] [EC:3.4.22.52]
122829987  calpain-1 catalytic subunit-like isoform X1 [KO:K01367] [EC:3.4.22.52]
122820130  capn1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
122842934  calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
122829988  calpain-2 catalytic subunit-like isoform X1 [KO:K03853] [EC:3.4.22.53]
122842935  calpain-2 catalytic subunit-like isoform X1 [KO:K03853] [EC:3.4.22.53]
122842936  calpain-2 catalytic subunit-like [KO:K03853] [EC:3.4.22.53]
122841528  E3 ubiquitin-protein ligase MARCHF6-like isoform X1 [KO:K10661] [EC:2.3.2.27]
122823889  E3 ubiquitin-protein ligase MARCHF6-like [KO:K10661] [EC:2.3.2.27]
122824865  ube2j1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
122834203  ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
122844743  ube2g1a; ubiquitin-conjugating enzyme E2 G1a [KO:K10575] [EC:2.3.2.23]
122828821  ubiquitin-conjugating enzyme E2 G1-like [KO:K10575] [EC:2.3.2.23]
122839741  ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
122837212  syvn1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
122843362  rnf5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
122847180  rnf185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
122841066  selenos; selenoprotein S [KO:K14025]
122824929  sel1l; LOW QUALITY PROTEIN: protein sel-1 homolog 1 [KO:K14026]
122835896  herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
122825123  E3 ubiquitin-protein ligase AMFR-like [KO:K10636] [EC:2.3.2.36]
122822739  stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
122846172  ubiquitin-conjugating enzyme E2 D2-like [KO:K06689] [EC:2.3.2.23]
122836765  ubiquitin-conjugating enzyme E2 2-like [KO:K06689] [EC:2.3.2.23]
122842124  ubiquitin-conjugating enzyme E2 D2-like isoform X1 [KO:K06689] [EC:2.3.2.23]
122837237  ube2d2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
122828059  ube2d4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
122842768  prkn; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
122822198  rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
122824392  cullin-1 [KO:K03347]
122844692  skp1; S-phase kinase-associated protein 1 [KO:K03094]
122834162  fbxo2; F-box only protein 2 [KO:K10099]
122839561  F-box only protein 6-like [KO:K10100]
122839573  F-box only protein 6-like [KO:K10100]
122839593  F-box only protein 6-like [KO:K10100]
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
gaf00510  N-Glycan biosynthesis
gaf03050  Proteasome
gaf03060  Protein export
gaf04210  Apoptosis
KO pathway
ko04141   
LinkDB

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