KEGG   PATHWAY: pteh04141
Entry
pteh04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Piliocolobus tephrosceles (Ugandan red Colobus)
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
pteh04141  Protein processing in endoplasmic reticulum
pteh04141

Other DBs
GO: 0030433 0034976
Organism
Piliocolobus tephrosceles (Ugandan red Colobus) [GN:pteh]
Gene
111550055  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
111544878  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
111536427  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
111555733  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
111543493  SEC62; translocation protein SEC62 isoform X1 [KO:K12275]
111538540  SEC63; translocation protein SEC63 homolog [KO:K09540]
111544912  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
111553303  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
111554711  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
111547733  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
111526538  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
111540297  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
111536055  MAGT1; magnesium transporter protein 1 [KO:K19478]
111547404  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
111547883  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
111524321  TMEM258; transmembrane protein 258 [KO:K26502]
111523414  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
111527064  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
111546418  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
111528592  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
111540228  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
111545930  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
111523209  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
111549931  DNAJC1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
111546581  dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
113219496  dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
111542796  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
111524308  HSP90B1; endoplasmin [KO:K09487]
111524349  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
111523875  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
111527410  CANX; calnexin [KO:K08054]
113219831  protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
111529489  protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
111523056  LOW QUALITY PROTEIN: calreticulin-like [KO:K08057]
111523829  CALR; calreticulin [KO:K08057]
111547282  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
111547665  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
111549644  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
111553018  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741] [EC:3.2.1.209]
111521583  LMAN2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
111546852  LMAN1; protein ERGIC-53 isoform X1 [KO:K10080]
111529664  LMAN1L; protein ERGIC-53-like [KO:K10081]
111554447  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
111529071  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
111537899  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
111546928  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
111538808  SEC31B; protein transport protein Sec31B [KO:K14005]
111549309  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
111524447  SEC23A; protein transport protein Sec23A [KO:K14006]
111546402  SEC23B; protein transport protein Sec23B [KO:K14006]
111529018  SEC24A; protein transport protein Sec24A [KO:K14007]
111537845  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
111547884  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
111547916  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
111551566  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
111546580  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
111546967  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
111548173  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
111536936  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
111534678  protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
111551492  PDIA4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
111531948  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
111537095  ERP29; endoplasmic reticulum resident protein 29 isoform X1 [KO:K09586]
111548613  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
111549442  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
111545240  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
111541793  OS9; protein OS-9 isoform X1 [KO:K10088]
111524886  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
111548610  SSR1; translocon-associated protein subunit alpha [KO:K13249]
111543947  SSR2; translocon-associated protein subunit beta [KO:K13250]
111550084  SSR3; translocon-associated protein subunit gamma [KO:K13251]
111533553  SSR4; translocon-associated protein subunit delta [KO:K04571]
111533617  uncharacterized protein LOC111533617 [KO:K14009]
111520932  TRAM1; translocating chain-associated membrane protein 1 isoform X1 [KO:K14010]
111547929  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
111550883  DERL1; derlin-1 isoform X1 [KO:K11519]
111521297  DERL2; derlin-2 isoform X1 [KO:K13989]
111522034  DERL3; derlin-3 isoform X2 [KO:K13989]
111534332  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
111527490  UBXN4; UBX domain-containing protein 4 [KO:K24348]
111554488  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
111521958  UBXN6; UBX domain-containing protein 6 [KO:K14011]
111544153  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
111548103  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
111525883  SVIP; small VCP/p97-interacting protein [KO:K14014]
111536776  VCP; transitional endoplasmic reticulum ATPase isoform X3 [KO:K13525]
113220256  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
111526723  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
113222861  heat shock 70 kDa protein 1 [KO:K03283]
111523910  heat shock 70 kDa protein 1 [KO:K03283]
111525033  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
111523908  heat shock 70 kDa protein 1 [KO:K03283]
111523909  heat shock 70 kDa protein 1-like [KO:K03283]
111540268  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
111529208  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
111524407  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
111554380  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
111548914  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
111537872  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
111520518  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
111554470  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
111549262  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
111543094  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
111538310  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
111549509  LOW QUALITY PROTEIN: heat shock protein HSP 90-alpha-like [KO:K04079]
111546252  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
111540468  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
111536814  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
111549775  BAG2; BAG family molecular chaperone regulator 2 isoform X1 [KO:K09556]
111531797  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
111526514  alpha-crystallin A chain [KO:K09541]
111540127  CRYAB; LOW QUALITY PROTEIN: alpha-crystallin B chain [KO:K09542]
111522718  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
111537218  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
111535998  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
111523830  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
111540937  UBQLN3; ubiquilin-3 [KO:K04523]
111543387  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
111543777  UBQLN4; ubiquilin-4 [KO:K04523]
111535902  UBQLN2; ubiquilin-2 [KO:K04523]
111539457  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
111531669  ATXN3L; ataxin-3-like protein [KO:K11863] [EC:3.4.22.-]
111538229  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
111544655  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
111528197  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
111519917  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
111542762  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
111548140  cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
111548991  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
111520035  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
111541810  DDIT3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452]
111528437  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
111525029  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
111528965  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
111539129  WFS1; wolframin [KO:K14020]
111552520  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
111534807  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
111525377  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
111542532  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
111553030  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
111525521  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
111522663  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
111530867  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
111535725  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
111554538  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
111520083  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
111542928  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
111522388  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
111537094  CAPN2; calpain-2 catalytic subunit isoform X1 [KO:K03853] [EC:3.4.22.53]
111540056  LOW QUALITY PROTEIN: caspase-12-like [KO:K04741] [EC:3.4.22.-]
111546805  MARCHF6; E3 ubiquitin-protein ligase MARCH6 isoform X1 [KO:K10661] [EC:2.3.2.27]
111538078  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
111526852  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
111527369  ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
111521243  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
111526916  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
111522389  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
111521835  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
111528963  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
111527355  SELENOS; selenoprotein S [KO:K14025]
111546432  SEL1L2; protein sel-1 homolog 2 isoform X2 [KO:K14026]
111538188  SEL1L; protein sel-1 homolog 1 [KO:K14026]
111541276  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
111541261  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
111524029  STUB1; E3 ubiquitin-protein ligase CHIP isoform X1 [KO:K09561] [EC:2.3.2.27]
111528414  ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
113219495  ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
111552308  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
111521925  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
111545552  ubiquitin-conjugating enzyme E2 D3-like [KO:K06689] [EC:2.3.2.23]
111547975  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
111550282  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
111532697  E3 ubiquitin-protein ligase RBX1-like [KO:K03868] [EC:2.3.2.32]
111548968  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
111551417  CUL1; cullin-1 [KO:K03347]
111529166  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
111526300  FBXO2; F-box only protein 2 [KO:K10099]
111526301  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
pteh00510  N-Glycan biosynthesis
pteh03050  Proteasome
pteh03060  Protein export
pteh04210  Apoptosis
pteh04930  Type II diabetes mellitus
pteh05010  Alzheimer disease
pteh05012  Parkinson disease
pteh05020  Prion disease
KO pathway
ko04141   
LinkDB

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