KEGG   PATHWAY: hmh04141
Entry
hmh04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Hylobates moloch (silvery gibbon)
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
hmh04141  Protein processing in endoplasmic reticulum
hmh04141

Other DBs
GO: 0030433 0034976
Organism
Hylobates moloch (silvery gibbon) [GN:hmh]
Gene
116473422  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
116466807  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
116477218  protein transport protein Sec61 subunit beta-like [KO:K09481]
116475325  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
116812663  protein transport protein Sec61 subunit gamma-like [KO:K07342]
116463773  protein transport protein Sec61 subunit gamma [KO:K07342]
116463806  protein transport protein Sec61 subunit gamma [KO:K07342]
116481228  SEC62; translocation protein SEC62 [KO:K12275]
116470584  SEC63; translocation protein SEC63 homolog [KO:K09540]
116466800  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
116461368  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
116484563  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
116813031  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
116477130  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A isoform X1 [KO:K07151] [EC:2.4.99.18]
116473709  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
116811824  MAGT1; magnesium transporter protein 1 [KO:K19478]
116472938  magnesium transporter protein 1-like isoform X1 [KO:K19478]
116473788  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
116478937  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X2 [KO:K26501]
116483880  oligosaccharyltransferase complex subunit OSTC-like [KO:K26501]
116473502  oligosaccharyltransferase complex subunit OSTC-like [KO:K26501]
116468866  TMEM258; transmembrane protein 258 [KO:K26502]
116483877  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
116478005  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
116461274  RRBP1; LOW QUALITY PROTEIN: ribosome-binding protein 1 [KO:K14000]
116471563  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
116477036  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
116475661  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
116479938  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
116477951  DNAJC1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
116467390  DNAJC3; dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
116810578  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
116478026  HSP90B1; endoplasmin [KO:K09487]
116469780  LOW QUALITY PROTEIN: endoplasmin-like [KO:K09487]
116467882  GANAB; neutral alpha-glucosidase AB [KO:K05546] [EC:3.2.1.207]
116478483  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
116471943  CANX; calnexin isoform X1 [KO:K08054]
116464358  protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
116464361  protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
116478555  CALR; calreticulin [KO:K08057]
116469545  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
116471498  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
116813096  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
116475908  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741] [EC:3.2.1.209]
116471896  LMAN2; vesicular integral-membrane protein VIP36 isoform X2 [KO:K10082]
116468509  LMAN1; protein ERGIC-53 [KO:K10080]
116481903  LMAN1L; LOW QUALITY PROTEIN: protein ERGIC-53-like [KO:K10081]
116480465  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
116458947  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
116471515  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
116466734  SEC13; protein SEC13 homolog isoform X2 [KO:K14004]
116485019  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
116466011  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
116460863  SEC23B; protein transport protein Sec23B [KO:K14006]
116456365  SEC23A; protein transport protein Sec23A [KO:K14006]
116459554  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
116471516  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
116462601  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
116463347  SEC24D; protein transport protein Sec24D isoform X3 [KO:K14007]
116467391  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X5 [KO:K11718] [EC:2.4.1.-]
116462871  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
116466688  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
116460948  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
116457395  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X3 [KO:K10086]
116483655  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
116814487  PDIA4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
116480230  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
116477904  ERP29; endoplasmic reticulum resident protein 29 isoform X1 [KO:K09586]
116461044  endoplasmic reticulum resident protein 29-like isoform X1 [KO:K09586]
116460203  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
116456342  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
116458116  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
116480852  OS9; protein OS-9 isoform X1 [KO:K10088]
116483203  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
116460152  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
116469296  SSR2; translocon-associated protein subunit beta [KO:K13250]
116463593  translocon-associated protein subunit gamma isoform X1 [KO:K13251]
116463611  translocon-associated protein subunit gamma isoform X1 [KO:K13251]
116812181  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
116812186  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
116463294  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
116813202  TRAM1; translocating chain-associated membrane protein 1 isoform X1 [KO:K14010]
116813560  DERL1; derlin-1 [KO:K11519]
116459163  DERL3; derlin-3 isoform X1 [KO:K13989]
116459492  DERL2; derlin-2 isoform X1 [KO:K13989]
116468895  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
116462923  UBXN4; UBX domain-containing protein 4 [KO:K24348]
116480390  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
116483539  UBXN6; UBX domain-containing protein 6 [KO:K14011]
116461926  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
116460872  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
116476422  SVIP; small VCP/p97-interacting protein [KO:K14014]
116475049  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
116483341  NPLOC4; nuclear protein localization protein 4 homolog isoform X2 [KO:K14015]
116459305  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
116477090  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
116460285  HSPA1B; heat shock 70 kDa protein 1B [KO:K03283]
116460286  HSPA1A; heat shock 70 kDa protein 1A [KO:K03283]
116460287  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
116473328  HSPA6; heat shock 70 kDa protein 6 [KO:K03283]
116459308  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
116463466  dnaJ homolog subfamily A member 1-like [KO:K09502]
116471221  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
116478599  DNAJB1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
116811118  LOW QUALITY PROTEIN: dnaJ homolog subfamily B member 1-like [KO:K09507]
116814334  LOW QUALITY PROTEIN: dnaJ homolog subfamily B member 1-like [KO:K09507]
116810265  DNAJB2; dnaJ homolog subfamily B member 2 isoform X2 [KO:K09508]
116459250  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
116460789  DNAJC5; dnaJ homolog subfamily C member 5 isoform X1 [KO:K09525]
116480476  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
116813238  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
116485178  HSP90AA1; heat shock protein HSP 90-alpha isoform X1 [KO:K04079]
116460213  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
116463739  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
116467101  HSPH1; heat shock protein 105 kDa isoform X2 [KO:K09485]
116458781  BAG1; BAG family molecular chaperone regulator 1 isoform X2 [KO:K09555]
116460140  BAG2; BAG family molecular chaperone regulator 2 isoform X2 [KO:K09556]
116479215  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
116482217  CRYAA; alpha-crystallin A chain isoform X1 [KO:K09541]
116476931  CRYAB; alpha-crystallin B chain isoform X1 [KO:K09542]
116457719  YOD1; ubiquitin thioesterase OTU1 isoform X1 [KO:K13719] [EC:3.1.2.-]
116458537  PLAA; phospholipase A-2-activating protein [KO:K14018]
116478556  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
116475512  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
116477340  UBQLN3; ubiquilin-3 [KO:K04523]
116469295  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
116811725  UBQLN2; ubiquilin-2 [KO:K04523]
116475191  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
116473729  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
116484858  ATXN3; ataxin-3 isoform X2 [KO:K11863] [EC:3.4.22.-]
116811002  ATXN3L; ataxin-3-like protein [KO:K11863] [EC:3.4.22.-]
116812897  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
116483180  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
116485158  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
116810621  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
116458613  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
116810327  LOW QUALITY PROTEIN: cyclic AMP-dependent transcription factor ATF-4-like [KO:K04374]
116478229  PPP1R15A; protein phosphatase 1 regulatory subunit 15A isoform X1 [KO:K14019]
116480869  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
116468493  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
116473320  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
116460313  cyclic AMP-dependent transcription factor ATF-6 beta-like [KO:K09049]
116456979  WFS1; wolframin [KO:K14020]
116470760  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
116811568  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
116459428  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
116458727  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
116475877  TRAF2; TNF receptor-associated factor 2 isoform X3 [KO:K03173] [EC:2.3.2.27]
116472781  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
116478375  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
116460769  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
116471970  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
116463128  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
116478225  BAX; apoptosis regulator BAX isoform X3 [KO:K02159]
116459971  BAK1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
116813623  LOW QUALITY PROTEIN: bcl-2 homologous antagonist/killer-like [KO:K14021]
116468992  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
116457885  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
116476195  LOW QUALITY PROTEIN: inactive caspase-12-like [KO:K04741] [EC:3.4.22.-]
116457073  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 [KO:K10661] [EC:2.3.2.27]
116469713  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X2 [KO:K10578] [EC:2.3.2.23]
116812769  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
116458580  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
116482560  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X2 [KO:K04555] [EC:2.3.2.23]
116468985  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X3 [KO:K10601] [EC:2.3.2.27]
116458825  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
116460320  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
116482248  SELENOS; selenoprotein S [KO:K14025]
116484941  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
116461522  SEL1L2; protein sel-1 homolog 2 isoform X6 [KO:K14026]
116471287  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
116471266  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
116462672  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
116458156  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
116461188  ubiquitin-conjugating enzyme E2 D3-like [KO:K06689] [EC:2.3.2.23]
116469510  ubiquitin-conjugating enzyme E2 D4-like [KO:K06689] [EC:2.3.2.23]
116811794  ubiquitin-conjugating enzyme E2 D2-like [KO:K06689] [EC:2.3.2.23]
116471577  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
116812503  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X3 [KO:K06689] [EC:2.3.2.23]
116482930  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X2 [KO:K06689] [EC:2.3.2.23]
116473652  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
116458544  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
116470866  LOW QUALITY PROTEIN: cullin-1 [KO:K03347]
116814485  CUL1; cullin-1 [KO:K03347]
116471506  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
116472739  S-phase kinase-associated protein 1-like [KO:K03094]
116812925  FBXO2; F-box only protein 2 isoform X1 [KO:K10099]
116812927  FBXO6; F-box only protein 6 [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
hmh00510  N-Glycan biosynthesis
hmh03050  Proteasome
hmh03060  Protein export
hmh04210  Apoptosis
hmh04930  Type II diabetes mellitus
hmh05010  Alzheimer disease
hmh05012  Parkinson disease
hmh05020  Prion disease
KO pathway
ko04141   
LinkDB

DBGET integrated database retrieval system