KEGG   PATHWAY: eai04141
Entry
eai04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Equus asinus (ass)
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
eai04141  Protein processing in endoplasmic reticulum
eai04141

Other DBs
GO: 0030433 0034976
Organism
Equus asinus (ass) [GN:eai]
Gene
106822100  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
106822213  AMFR; LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
106822225  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
106822285  [KO:K03283]
106822332  [KO:K06689] [EC:2.3.2.23]
106822892  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
106822991  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
106823060  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
106823842  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
106823874  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
106824450  UBXN8; UBX domain-containing protein 8 [KO:K24351]
106825061  [KO:K03283]
106825307  CRYAA; alpha-crystallin A chain [KO:K09541]
106825388  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
106825623  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
106825702  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
106825705  DERL2; derlin-2 isoform X1 [KO:K13989]
106825792  SEL1L2; protein sel-1 homolog 2 isoform X1 [KO:K14026]
106825998  [KO:K08057]
106826597  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
106826615  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
106826662  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
106826835  GANAB; neutral alpha-glucosidase AB isoform X2 [KO:K05546] [EC:3.2.1.207]
106826841  UBXN1; UBX domain-containing protein 1 [KO:K24348]
106826895  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
106826908  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
106827154  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
106827572  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
106827800  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
106827898  MAGT1; magnesium transporter protein 1 [KO:K19478]
106828477  UBQLN4; LOW QUALITY PROTEIN: ubiquilin-4 [KO:K04523]
106828478  SSR2; translocon-associated protein subunit beta [KO:K13250]
106828501  KRTCAP2; LOW QUALITY PROTEIN: keratinocyte-associated protein 2 [KO:K28202]
106828754  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
106828766  RRBP1; LOW QUALITY PROTEIN: ribosome-binding protein 1 [KO:K14000]
106829004  [KO:K26502]
106829117  DERL1; derlin-1 isoform X1 [KO:K11519]
106829535  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
106829699  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
106829804  SEC24B; LOW QUALITY PROTEIN: protein transport protein Sec24B [KO:K14007]
106829809  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
106829892  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
106830213  [KO:K04523]
106830488  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
106830620  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
106830721  ERO1A; ERO1-like protein alpha isoform X1 [KO:K10950] [EC:1.8.4.-]
106830826  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
106830999  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
106831009  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
106831022  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
106831112  HSPA6; heat shock 70 kDa protein 6 [KO:K03283]
106831122  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
106831267  LMAN1L; protein ERGIC-53-like isoform X1 [KO:K10081]
106831311  [KO:K14014]
106831384  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
106831751  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
106832088  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
106832092  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
106832284  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
106832376  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
106832535  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
106832775  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
106832883  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
106833003  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
106833014  OS9; protein OS-9 isoform X1 [KO:K10088]
106833069  SEC23A; protein transport protein Sec23A [KO:K14006]
106833590  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
106833593  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
106833689  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
106834321  DNAJC5B; dnaJ homolog subfamily C member 5B isoform X2 [KO:K09525]
106834472  SEC61A1; LOW QUALITY PROTEIN: protein transport protein Sec61 subunit alpha [KO:K10956]
106834477  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 isoform X1 [KO:K12666]
106834546  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
106834586  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
106834656  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
106834675  SEC62; translocation protein SEC62 [KO:K12275]
106834678  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
106834695  ERO1B; ERO1-like protein beta isoform X2 [KO:K10976] [EC:1.8.4.-]
106834795  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
106835017  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
106835057  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
106835342  SSR3; translocon-associated protein subunit gamma isoform X1 [KO:K13251]
106835465  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
106835512  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
106835534  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
106835569  ERN1; LOW QUALITY PROTEIN: serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
106835629  SEC13; protein SEC13 homolog isoform X2 [KO:K14004]
106835703  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X2 [KO:K01230] [EC:3.2.1.113]
106835961  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
106836179  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
106836298  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
106836525  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
106836542  UBXN4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
106836671  [KO:K10100]
106836672  FBXO2; F-box only protein 2 [KO:K10099]
106836691  UBE4B; ubiquitin conjugation factor E4 B isoform X4 [KO:K10597] [EC:2.3.2.27]
106837002  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
106837031  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
106837040  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
106837192  MAP3K5; LOW QUALITY PROTEIN: mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
106837271  [KO:K10085]
106837654  [KO:K04741] [EC:3.4.22.-]
106837951  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
106838262  PPP1R15A; LOW QUALITY PROTEIN: protein phosphatase 1 regulatory subunit 15A [KO:K14019]
106838268  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
106838571  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
106838735  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
106838958  PLAA; phospholipase A-2-activating protein [KO:K14018]
106838972  UBQLN2; ubiquilin-2 [KO:K04523]
106839223  SIL1; nucleotide exchange factor SIL1 isoform X2 [KO:K14001]
106839421  MAPK10; mitogen-activated protein kinase 10 isoform X2 [KO:K04440] [EC:2.7.11.24]
106839467  SELENOS; selenoprotein S isoform X2 [KO:K14025]
106839639  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
106839943  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
106840022  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
106840211  CRYAB; alpha-crystallin B chain [KO:K09542]
106840296  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
106840424  SEC23B; protein transport protein Sec23B [KO:K14006]
106840554  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
106840668  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
106840931  HSPBP1; hsp70-binding protein 1 [KO:K09562]
106841452  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
106841465  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
106841535  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
106841893  SEC24D; protein transport protein Sec24D [KO:K14007]
106841977  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
106842050  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
106842121  CUL1; cullin-1 [KO:K03347]
106842260  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
106842437  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
106842455  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X4 [KO:K03868] [EC:2.3.2.32]
106842495  LMAN1; protein ERGIC-53 isoform X1 [KO:K10080]
106842608  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
106842874  [KO:K10839]
106842923  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
106842972  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X2 [KO:K04555] [EC:2.3.2.23]
106843315  WFS1; LOW QUALITY PROTEIN: wolframin [KO:K14020]
106843537  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
106843718  CANX; calnexin [KO:K08054]
106843729  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
106844304  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
106844336  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
106844337  CALR; calreticulin [KO:K08057]
106844503  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
106844808  [KO:K13989]
106844926  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
106845025  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
106845238  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
106845522  [KO:K23741]
106845532  TRAF2; TNF receptor-associated factor 2 isoform X4 [KO:K03173] [EC:2.3.2.27]
106845601  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
106845789  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
106845915  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
106846045  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
106846227  DNAJC3; dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
106846228  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
106846313  DNAJC1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
106846426  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
106846601  PRKCSH; glucosidase 2 subunit beta isoform X2 [KO:K08288]
106846750  SEC31B; protein transport protein Sec31B isoform X2 [KO:K14005]
106846786  HSP90B1; endoplasmin [KO:K09487]
106846824  CKAP4; LOW QUALITY PROTEIN: cytoskeleton-associated protein 4 [KO:K13999]
106846998  [KO:K13525]
106847228  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
106847255  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
106847464  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X3 [KO:K10661] [EC:2.3.2.27]
106847589  TMEM258; transmembrane protein 258 [KO:K26502]
106848247  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
106848452  EDEM1; LOW QUALITY PROTEIN: ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
106848453  SEC63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
123280223  [KO:K09502]
123282460  [KO:K06689] [EC:2.3.2.23]
123282475  [KO:K06689] [EC:2.3.2.23]
123282546  [KO:K06689] [EC:2.3.2.23]
123282797  [KO:K03283]
123288213  [KO:K03283]
123288236  [KO:K13989]
123289508  [KO:K23741]
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
eai00510  N-Glycan biosynthesis
eai03050  Proteasome
eai03060  Protein export
eai04210  Apoptosis
eai04930  Type II diabetes mellitus
eai05010  Alzheimer disease
eai05012  Parkinson disease
eai05020  Prion disease
KO pathway
ko04141   

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