KEGG   PATHWAY: pmur04141
Entry
pmur04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Protobothrops mucrosquamatus (Taiwan habu)
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
pmur04141  Protein processing in endoplasmic reticulum
pmur04141

Other DBs
GO: 0030433 0034976
Organism
Protobothrops mucrosquamatus (Taiwan habu) [GN:pmur]
Gene
107297193  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
107294279  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
107291672  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
107284698  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
107290253  SEC62; translocation protein SEC62 [KO:K12275]
107292061  SEC63; translocation protein SEC63 homolog [KO:K09540]
107292969  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
107287251  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
107287152  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
107297526  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
107296401  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
107284379  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
107293036  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B-like [KO:K07151] [EC:2.4.99.18]
107284623  MAGT1; magnesium transporter protein 1 [KO:K19478]
107287928  TUSC3; tumor suppressor candidate 3 [KO:K19478]
107282996  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
107287962  TMEM258; transmembrane protein 258 [KO:K26502]
107292508  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
107289581  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
107299533  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
107289866  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
107284796  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
107296774  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
107285871  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
107283630  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
107290355  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
107291315  dnaJ homolog subfamily C member 3-like [KO:K09523]
107292634  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
107294617  HSP90B1; endoplasmin [KO:K09487]
107292414  GANAB; neutral alpha-glucosidase AB [KO:K05546] [EC:3.2.1.207]
107285142  PRKCSH; glucosidase 2 subunit beta [KO:K08288]
107296594  CANX; calnexin isoform X1 [KO:K08054]
107289787  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
107283024  calreticulin [KO:K08057]
107282162  CALR3; calreticulin-3 [KO:K08057]
107285132  CALR; calreticulin [KO:K08057]
107295591  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
107293110  mannosyl-oligosaccharide 1,2-alpha-mannosidase IC-like [KO:K01230] [EC:3.2.1.113]
107299749  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
107285786  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
107295058  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
107295192  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
107290862  LMAN1; protein ERGIC-53 [KO:K10080]
107286450  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
107294962  SAR1B; GTP-binding protein SAR1b isoform X1 [KO:K07953] [EC:3.6.5.-]
107296304  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
107297846  SEC13; protein SEC13 homolog [KO:K14004]
107303172  SEC31A; protein transport protein Sec31A [KO:K14005]
107295957  SEC23A; protein transport protein Sec23A [KO:K14006]
107284462  SEC23B; protein transport protein Sec23B [KO:K14006]
107294985  SEC24A; protein transport protein Sec24A [KO:K14007]
107298833  SEC24C; protein transport protein Sec24C [KO:K14007]
107294083  SEC24D; protein transport protein Sec24D [KO:K14007]
107283192  SEC24B; protein transport protein Sec24B [KO:K14007]
107290356  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
107287665  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
107298918  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
107285582  ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
107288064  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
107295827  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
107292484  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
107294779  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
107286970  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
107282994  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
107289096  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
107297851  ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
107288515  OS9; protein OS-9 [KO:K10088]
107285495  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
107283001  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
107300395  translocon-associated protein subunit beta [KO:K13250]
107288052  translocon-associated protein subunit beta [KO:K13250]
107286945  SSR3; translocon-associated protein subunit gamma [KO:K13251]
107284225  SSR4; translocon-associated protein subunit delta [KO:K04571]
107286705  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
107290246  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
107290353  translocating chain-associated membrane protein 1-like 1 [KO:K14010]
107295160  DERL1; derlin-1 [KO:K11519]
107296913  DERL2; derlin-2 [KO:K13989]
107284391  UBXN4; UBX domain-containing protein 4 [KO:K24348]
107292402  UBX domain-containing protein 1-like [KO:K24348]
107293405  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
107297949  UBXN6; UBX domain-containing protein 6 [KO:K14011]
107299770  NSFL1 cofactor p47 [KO:K14012]
107299780  NSFL1 cofactor p47-like [KO:K14012]
107293016  SVIP; small VCP/p97-interacting protein [KO:K14014]
107299182  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
107293242  transitional endoplasmic reticulum ATPase [KO:K13525]
107286772  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
107293417  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
107289938  heat shock 70 kDa protein 1B-like [KO:K03283]
107289939  heat shock 70 kDa protein II-like [KO:K03283]
107292332  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
107293382  heat shock cognate 71 kDa protein [KO:K03283]
107298215  dnaJ homolog subfamily A member 1-like [KO:K09502]
107285613  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
107290112  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
107292472  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
107288116  dnaJ homolog subfamily B member 2-like [KO:K09508]
107288121  dnaJ homolog subfamily B member 6-like [KO:K09508]
107288101  DNAJB12; dnaJ homolog subfamily B member 12 [KO:K09518]
107293440  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
107288951  DNAJC5B; dnaJ homolog subfamily C member 5B isoform X1 [KO:K09525]
107286446  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
107294792  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
107295973  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
107290574  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
107286804  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
107289430  BAG1; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555]
107295497  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
107282482  HSPBP1; hsp70-binding protein 1 [KO:K09562]
107295079  alpha-crystallin A chain [KO:K09541]
107284519  CRYAB; alpha-crystallin B chain [KO:K09542]
107287998  HSPB6; heat shock protein beta-6 [KO:K09542]
107288375  YOD1; ubiquitin thioesterase OTU1 isoform X1 [KO:K13719] [EC:3.1.2.-]
107290678  PLAA; phospholipase A-2-activating protein [KO:K14018]
107285133  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
107292291  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
107298537  ubiquilin-3-like [KO:K04523]
107298538  ubiquilin-1-like [KO:K04523]
107297865  ubiquilin-3-like [KO:K04523]
107300921  ubiquilin-4-like [KO:K04523]
107290912  UBQLN4; ubiquilin-4 [KO:K04523]
107284448  UBQLN1; ubiquilin-1 [KO:K04523]
107297866  LOW QUALITY PROTEIN: ubiquilin-1-like [KO:K04523]
114923499  ubiquilin-1-like [KO:K04523]
107283763  NGLY1; LOW QUALITY PROTEIN: peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
107297374  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
107292359  UBE4B; ubiquitin conjugation factor E4 B isoform X3 [KO:K10597] [EC:2.3.2.27]
107302385  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
107285843  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
107286580  NFE2L2; nuclear factor erythroid 2-related factor 2 [KO:K05638]
107288132  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
107282487  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
107293466  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
107283580  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
107302856  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
107298366  cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
107299172  WFS1; wolframin [KO:K14020]
107293778  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
107286126  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
107287284  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
107284852  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
107299991  serine/threonine-protein kinase/endoribonuclease IRE1-like [KO:K08852] [EC:2.7.11.1 3.1.26.-]
107296550  TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
107302543  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
107282238  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
107291150  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
107294632  MAPK9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
107293954  MAPK8; mitogen-activated protein kinase 8 isoform X3 [KO:K04440] [EC:2.7.11.24]
107303183  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
107295492  apoptosis regulator BAX-like [KO:K02159]
107282460  apoptosis regulator BAX [KO:K02159]
107282547  apoptosis regulator BAX-like [KO:K02159]
107287068  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
107294458  calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
107294378  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
107288074  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
107292210  MARCH6; E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
107296171  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
107287524  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
107292004  UBE2G1; ubiquitin-conjugating enzyme E2 G1 isoform X1 [KO:K10575] [EC:2.3.2.23]
107285876  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
107294375  SYVN1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
107301084  E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
107296707  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
107293503  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
107283883  SELENOS; selenoprotein S [KO:K14025]
107296453  SEL1L; protein sel-1 homolog 1 [KO:K14026]
107293228  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
107293777  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
107296064  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
107290384  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
107299024  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
107290002  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
107284471  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
107295102  PRKN; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
107285413  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
107288848  cullin-1 [KO:K03347]
107294918  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
107299410  F-box only protein 2 [KO:K10099]
107294609  F-box only protein 44 [KO:K10100]
107299412  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
pmur00510  N-Glycan biosynthesis
pmur03050  Proteasome
pmur03060  Protein export
pmur04210  Apoptosis
KO pathway
ko04141   
LinkDB

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