KEGG   PATHWAY: muo04141
Entry
muo04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Microcaecilia unicolor
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
muo04141  Protein processing in endoplasmic reticulum
muo04141

Other DBs
GO: 0030433 0034976
Organism
Microcaecilia unicolor [GN:muo]
Gene
115471771  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
115471490  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
115470526  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
115478419  SEC62; translocation protein SEC62 isoform X1 [KO:K12275]
115467313  SEC63; translocation protein SEC63 homolog [KO:K09540]
115472032  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
115475972  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
115457789  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
115457791  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
115456817  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
115481532  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
115471957  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
115473795  MAGT1; magnesium transporter protein 1 [KO:K19478]
115461549  TUSC3; tumor suppressor candidate 3 [KO:K19478]
115462531  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
115468972  TMEM258; transmembrane protein 258 [KO:K26502]
115461222  MOGS; mannosyl-oligosaccharide glucosidase isoform X1 [KO:K01228] [EC:3.2.1.106]
115477949  CKAP4; LOW QUALITY PROTEIN: cytoskeleton-associated protein 4 [KO:K13999]
115465633  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
115476562  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
115481868  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
115472906  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
115474179  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
115467911  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
115469081  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
115457653  dnaJ homolog subfamily C member 3-like [KO:K09523]
115474132  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
115477360  HSP90B1; endoplasmin [KO:K09487]
115479542  GANAB; LOW QUALITY PROTEIN: neutral alpha-glucosidase AB [KO:K05546] [EC:3.2.1.207]
115466022  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
115475509  CANX; calnexin isoform X1 [KO:K08054]
115459843  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
115480948  CALR3; calreticulin-3 [KO:K08057]
115471964  calreticulin-like [KO:K08057]
115466034  CALR; calreticulin [KO:K08057]
115480256  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
115470665  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
115466959  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
115472697  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
115476524  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
115462909  LMAN1; protein ERGIC-53 isoform X1 [KO:K10080]
115457094  protein ERGIC-53-like [KO:K10080]
115466990  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
115476577  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
115470381  SAR1A; GTP-binding protein SAR1a isoform X1 [KO:K07953] [EC:3.6.5.-]
115471642  SEC13; protein SEC13 homolog [KO:K14004]
115461067  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
115470390  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
115477307  protein transport protein Sec23A [KO:K14006]
115465623  SEC23B; protein transport protein Sec23B [KO:K14006]
115461175  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
115461751  SEC24D; protein transport protein Sec24D [KO:K14007]
115476296  SEC24A; LOW QUALITY PROTEIN: protein transport protein Sec24A [KO:K14007]
115470337  SEC24C; protein transport protein Sec24C [KO:K14007]
115469080  UGGT2; LOW QUALITY PROTEIN: UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
115472106  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
115463190  ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
115471788  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
115471540  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
115470763  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
115467142  PDIA6; LOW QUALITY PROTEIN: protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
115480388  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
115473833  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
115477289  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
115465831  OS9; protein OS-9 isoform X1 [KO:K10088]
115465402  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
115473751  SSR1; translocon-associated protein subunit alpha [KO:K13249]
115457514  SSR2; translocon-associated protein subunit beta isoform X1 [KO:K13250]
115478536  SSR3; translocon-associated protein subunit gamma [KO:K13251]
115463509  translocon-associated protein subunit delta-like [KO:K04571]
115463510  translocon-associated protein subunit delta-like [KO:K04571]
115463503  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
115476290  LOW QUALITY PROTEIN: translocating chain-associated membrane protein 1-like [KO:K14010]
115478145  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
115480171  DERL1; derlin-1 [KO:K11519]
115480060  DERL3; derlin-3 [KO:K13989]
115480235  UBXN1; UBX domain-containing protein 1 [KO:K24348]
115475160  UBXN4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
115467109  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
115480323  UBXN6; UBX domain-containing protein 6 [KO:K14011]
115463747  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
115475694  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
115468750  SVIP; small VCP/p97-interacting protein [KO:K14014]
115461786  transitional endoplasmic reticulum ATPase-like [KO:K13525]
115462810  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
115466101  transitional endoplasmic reticulum ATPase-like [KO:K13525]
115459034  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
115480079  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
115475428  heat shock cognate 71 kDa protein-like [KO:K03283]
115482041  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
115466274  heat shock 70 kDa protein 1B [KO:K03283]
115463634  DNAJA1; LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 1 [KO:K09502]
115457917  dnaJ homolog subfamily A member 1-like [KO:K09502]
115470820  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
115466097  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
115474898  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
115470376  DNAJB12; dnaJ homolog subfamily B member 12 [KO:K09518]
115475701  DNAJC5; dnaJ homolog subfamily C member 5 isoform X1 [KO:K09525]
115477976  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
115466984  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
115477177  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
115465334  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
115458986  heat shock protein HSP 90-beta-like [KO:K04079]
115468562  HSPH1; heat shock protein 105 kDa [KO:K09485]
115471072  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
115465218  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
115466547  HSPBP1; hsp70-binding protein 1 [KO:K09562]
115481543  CRYAB; alpha-crystallin B chain [KO:K09542]
115476396  HSPB6; heat shock protein beta-6 [KO:K09542]
115481519  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
115463542  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
115463624  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
115466035  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
115463305  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
115457503  UBQLN4; ubiquilin-4 [KO:K04523]
115471838  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
115459116  peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase-like [KO:K01456] [EC:3.5.1.52]
115477371  ATXN3; LOW QUALITY PROTEIN: ataxin-3 [KO:K11863] [EC:3.4.22.-]
115482562  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
115461322  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
115477226  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
115475110  NFE2L2; LOW QUALITY PROTEIN: nuclear factor erythroid 2-related factor 2 [KO:K05638]
115473430  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
115465843  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
115472967  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
115471968  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
115466197  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
115461402  WFS1; wolframin isoform X1 [KO:K14020]
115470543  MBTPS1; LOW QUALITY PROTEIN: membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
115469519  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
115479710  XBP1; X-box-binding protein 1 isoform X1 [KO:K09027]
115475539  ERN2; serine/threonine-protein kinase/endoribonuclease IRE2 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
115473455  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
115472619  TRAF2; LOW QUALITY PROTEIN: TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
115479652  MAP3K6; LOW QUALITY PROTEIN: mitogen-activated protein kinase kinase kinase 6 [KO:K04426] [EC:2.7.11.25]
115466925  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
115466111  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
115461062  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
115476478  MAPK9; LOW QUALITY PROTEIN: mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
115470198  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
115466621  BAX; apoptosis regulator BAX [KO:K02159]
115481830  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
115480899  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
115465329  CAPN11; calpain-11 [KO:K01367] [EC:3.4.22.52]
115481010  calpain-12-like [KO:K03853] [EC:3.4.22.53]
115465480  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
115471506  E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
115471567  E3 ubiquitin-protein ligase MARCH6-like [KO:K10661] [EC:2.3.2.27]
115467276  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
115482626  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
115456648  UBE2G1; ubiquitin-conjugating enzyme E2 G1 isoform X1 [KO:K10575] [EC:2.3.2.23]
115474467  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
115480053  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
115466186  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
115479467  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
115459915  SELENOS; selenoprotein S [KO:K14025]
115459918  selenoprotein S-like [KO:K14025]
115477522  SEL1L; protein sel-1 homolog 1 [KO:K14026]
115465685  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
115470505  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
115470492  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
115475610  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
115461152  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
115469278  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
115470151  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
115460141  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
115470481  CUL1; cullin-1 [KO:K03347]
115475708  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
115456717  FBXO2; F-box only protein 2 [KO:K10099]
115456716  F-box only protein 6-like isoform X1 [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
muo00510  N-Glycan biosynthesis
muo03050  Proteasome
muo03060  Protein export
muo04210  Apoptosis
KO pathway
ko04141   
LinkDB

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