KEGG   PATHWAY: ptr04141
Entry
ptr04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Pan troglodytes (chimpanzee)
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
ptr04141  Protein processing in endoplasmic reticulum
ptr04141

Other DBs
GO: 0030433 0034976
Organism
Pan troglodytes (chimpanzee) [GN:ptr]
Gene
100608041  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
100608599  [KO:K04079]
100608758  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
100610529  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
100610792  SEC31B; LOW QUALITY PROTEIN: protein transport protein Sec31B [KO:K14005]
100610941  UBQLN3; ubiquilin-3 [KO:K04523]
100613210  RRBP1; ribosome-binding protein 1 isoform X6 [KO:K14000]
100614603  SSR1; translocon-associated protein subunit alpha [KO:K13249]
100615379  [KO:K14016]
100615889  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
101057367  [KO:K04523]
107966147  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X2 [KO:K26501]
107967332  [KO:K09481]
107967560  [KO:K12668]
107973291  [KO:K03283]
107975108  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X3 [KO:K09049]
107975372  [KO:K26501]
112205859  HSPA6; heat shock 70 kDa protein 6 [KO:K03283]
129136056  [KO:K26501]
129138867  [KO:K08056] [EC:5.3.4.1]
129143655  [KO:K26501]
134809375  ATXN3L; ataxin-3-like protein [KO:K11863] [EC:3.4.22.-]
387278  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
450305  SEC61A2; protein transport protein Sec61 subunit alpha isoform X3 [KO:K10956]
450344  DNAJC1; DnaJ homolog subfamily C member 1 isoform X2 [KO:K09521]
450445  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
450525  SEC24C; protein transport protein Sec24C isoform X2 [KO:K14007]
450887  DNAJB12; DnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
451082  SVIP; small VCP/p97-interacting protein [KO:K14014]
451258  GANAB; neutral alpha-glucosidase AB isoform X2 [KO:K05546] [EC:3.2.1.207]
451261  UBXN1; UBX domain-containing protein 1 isoform X2 [KO:K24348]
451317  CAPN1; calpain-1 catalytic subunit isoform X1 [KO:K01367] [EC:3.4.22.52]
451543  CRYAB; alpha-crystallin B chain [KO:K09542]
451600  HYOU1; hypoxia up-regulated protein 1 isoform X4 [KO:K09486]
451629  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
451648  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A isoform X1 [KO:K07151] [EC:2.4.99.18]
452017  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
452022  OS9; protein OS-9 isoform X2 [KO:K10088]
452187  HSP90B1; endoplasmin isoform X2 [KO:K09487]
452522  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
452628  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X2 [KO:K11718] [EC:2.4.1.-]
452783  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
452871  SEC23A; protein transport protein Sec23A [KO:K14006]
452908  ERO1A; ERO1-like protein alpha isoform X2 [KO:K10950] [EC:1.8.4.-]
452980  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
453080  SEL1L; protein sel-1 homolog 1 isoform X2 [KO:K14026]
453691  SELENOS; selenoprotein S [KO:K14025]
453803  STUB1; E3 ubiquitin-protein ligase CHIP isoform X1 [KO:K09561] [EC:2.3.2.27]
454109  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X5 [KO:K14027]
454769  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
454960  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
454970  P4HB; protein disulfide-isomerase precursor [KO:K09580] [EC:5.3.4.1]
455251  DERL2; derlin-2 isoform X1 [KO:K13989]
455448  LMAN1; protein ERGIC-53 [KO:K10080]
455604  UBXN6; UBX domain-containing protein 6 isoform X2 [KO:K14011]
455731  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
455756  CALR; calreticulin [KO:K08057]
456191  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
456299  HSPBP1; hsp70-binding protein 1 isoform X2 [KO:K09562]
456748  [KO:K19478]
457015  [KO:K09487]
457159  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
457188  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
457379  SSR2; translocon-associated protein subunit beta [KO:K13250]
457380  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
457461  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X3 [KO:K09054]
457578  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X2 [KO:K10086]
457765  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
457924  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
457951  FBXO2; F-box only protein 2 [KO:K10099]
458197  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
458228  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
458423  DNAJC5; DnaJ homolog subfamily C member 5 [KO:K09525]
458432  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
458589  CRYAA; alpha-crystallin A chain isoform X2 [KO:K09541]
458650  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X2 [KO:K14016]
458735  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
458768  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
458860  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X2 [KO:K03868] [EC:2.3.2.32]
459094  PREB; prolactin regulatory element-binding protein isoform X3 [KO:K14003]
459388  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
459509  PDIA6; protein disulfide-isomerase A6 precursor [KO:K09584] [EC:5.3.4.1]
459600  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
459632  UBXN4; UBX domain-containing protein 4 [KO:K24348]
459799  DNAJC10; DnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
460167  SEC13; protein SEC13 homolog [KO:K14004]
460233  NGLY1; peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase isoform X2 [KO:K01456] [EC:3.5.1.52]
460669  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
460673  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 precursor [KO:K12666]
460799  SSR3; translocon-associated protein subunit gamma isoform X1 [KO:K13251]
460911  DNAJB11; DnaJ homolog subfamily B member 11 [KO:K09517]
461100  WFS1; wolframin isoform X1 [KO:K14020]
461210  SEC31A; protein transport protein Sec31A isoform X13 [KO:K14005]
461358  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
461431  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
461660  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
461722  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
461941  [KO:K06689] [EC:2.3.2.23]
462063  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
462069  SAR1B; GTP-binding protein SAR1b isoform X1 [KO:K07953] [EC:3.6.5.-]
462108  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X2 [KO:K06689] [EC:2.3.2.23]
462302  LMAN2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
462330  CANX; calnexin isoform X2 [KO:K08054]
462336  MAPK9; mitogen-activated protein kinase 9 isoform X2 [KO:K04440] [EC:2.7.11.24]
462616  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
462797  BAG2; BAG family molecular chaperone regulator 2 isoform X1 [KO:K09556]
462883  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X2 [KO:K10578] [EC:2.3.2.23]
462915  SEC63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
463023  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
463148  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
463379  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X5 [KO:K06689] [EC:2.3.2.23]
463446  [KO:K03094]
463821  CUL1; cullin-1 isoform X1 [KO:K03347]
463822  PDIA4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
464002  TUSC3; tumor suppressor candidate 3 isoform X3 [KO:K19478]
464123  [KO:K10666] [EC:2.3.2.27]
464227  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
464550  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
464733  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
464876  TRAF2; TNF receptor-associated factor 2 isoform X3 [KO:K03173] [EC:2.3.2.27]
465026  PLAA; phospholipase A-2-activating protein isoform X2 [KO:K14018]
465041  BAG1; BAG family molecular chaperone regulator 1 isoform X3 [KO:K09555]
465532  MBTPS2; membrane-bound transcription factor site-2 protease isoform X2 [KO:K07765] [EC:3.4.24.85]
465726  MAGT1; magnesium transporter protein 1 [KO:K19478]
465928  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
466660  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
466766  CASP12; inactive caspase-12 isoform X1 [KO:K04741] [EC:3.4.22.-]
467826  LMAN1L; LOW QUALITY PROTEIN: protein ERGIC-53-like [KO:K10081]
468050  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
468747  DNAJB1; DnaJ homolog subfamily B member 1 [KO:K09507]
469659  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
469720  ERO1B; ERO1-like protein beta precursor [KO:K10976] [EC:1.8.4.-]
469799  FBXO6; F-box only protein 6 [KO:K10100]
469859  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
469885  SEL1L2; protein sel-1 homolog 2 isoform X15 [KO:K14026]
470326  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
470375  ERLEC1; endoplasmic reticulum lectin 1 precursor [KO:K14008]
471107  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
471289  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
471787  SEC24A; protein transport protein Sec24A isoform X2 [KO:K14007]
471967  HSPA1L; heat shock 70 kDa protein 1-like isoform X1 [KO:K03283]
472599  [KO:K03283]
473012  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
473099  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X2 [KO:K23741]
473274  DNAJA2; DnaJ homolog subfamily A member 2 [KO:K09503]
474042  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X2 [KO:K04555] [EC:2.3.2.23]
735643  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
736491  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
736800  DNAJC3; DnaJ homolog subfamily C member 3 [KO:K09523]
737497  HSP90AB1; heat shock protein HSP 90-beta isoform X1 [KO:K04079]
738376  ERP29; endoplasmic reticulum resident protein 29 isoform X1 [KO:K09586]
738409  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
738729  ATXN3; ataxin-3 isoform X6 [KO:K11863] [EC:3.4.22.-]
738935  BCAP31; B-cell receptor-associated protein 31 isoform X1 [KO:K14009]
739125  TMEM258; transmembrane protein 258 [KO:K26502]
740472  MOGS; mannosyl-oligosaccharide glucosidase isoform X1 [KO:K01228] [EC:3.2.1.106]
741033  [KO:K03094]
741108  DNAJC5G; DnaJ homolog subfamily C member 5G isoform X2 [KO:K09525]
741350  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
742622  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
742720  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
742723  PDIA3; protein disulfide-isomerase A3 precursor [KO:K08056] [EC:5.3.4.1]
742975  HSPA4L; heat shock 70 kDa protein 4L isoform X2 [KO:K09485]
743199  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X3 [KO:K04431] [EC:2.7.12.2]
743367  DNAJB2; DnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
743819  UBQLN2; ubiquilin-2 [KO:K04523]
743883  HSP90AA1; heat shock protein HSP 90-alpha isoform X1 [KO:K04079]
744068  DNAJC5B; DnaJ homolog subfamily C member 5B isoform X1 [KO:K09525]
744092  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
744481  SEC62; translocation protein SEC62 isoform X2 [KO:K12275]
744513  ATF4; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374]
745118  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
745141  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X2 [KO:K06689] [EC:2.3.2.23]
745166  HSPA1B; heat shock 70 kDa protein 1B [KO:K03283]
745946  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
746396  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
746627  DNAJA1; DnaJ homolog subfamily A member 1 [KO:K09502]
747093  BAX; apoptosis regulator BAX isoform X4 [KO:K02159]
747196  DERL3; derlin-3 isoform X5 [KO:K13989]
747459  SIL1; nucleotide exchange factor SIL1 precursor [KO:K14001]
747490  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
747589  SEC61B; protein transport protein Sec61 subunit beta isoform X2 [KO:K09481]
748740  [KO:K03283]
750513  UBE2G1; ubiquitin-conjugating enzyme E2 G1 isoform X2 [KO:K10575] [EC:2.3.2.23]
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
ptr00510  N-Glycan biosynthesis
ptr03050  Proteasome
ptr03060  Protein export
ptr04210  Apoptosis
ptr04930  Type II diabetes mellitus
ptr05010  Alzheimer disease
ptr05012  Parkinson disease
ptr05020  Prion disease
KO pathway
ko04141   

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