KEGG   PATHWAY: oga04141
Entry
oga04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Otolemur garnettii (small-eared galago)
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
oga04141  Protein processing in endoplasmic reticulum
oga04141

Other DBs
GO: 0030433 0034976
Organism
Otolemur garnettii (small-eared galago) [GN:oga]
Gene
100953274  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
100964664  SEC61A2; protein transport protein Sec61 subunit alpha isoform X2 [KO:K10956]
100953952  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
111725386  protein transport protein Sec61 gamma-1 subunit-like [KO:K07342]
111726613  protein transport protein Sec61 subunit gamma-like [KO:K07342]
100960473  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
100953164  SEC62; translocation protein SEC62 [KO:K12275]
100952871  SEC63; translocation protein SEC63 homolog [KO:K09540]
100955573  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
100940387  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
100956353  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
100954876  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
100941691  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
100961680  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
100952143  MAGT1; magnesium transporter protein 1 [KO:K19478]
100965119  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
100959781  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
100955275  TMEM258; transmembrane protein 258 [KO:K26502]
100941152  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
100945311  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
100941633  RRBP1; ribosome-binding protein 1 isoform X3 [KO:K14000]
100959806  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
100959566  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
100946481  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
100959666  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
100944495  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
100955320  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
100949027  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
100949628  HSP90B1; endoplasmin [KO:K09487]
100947385  GANAB; neutral alpha-glucosidase AB [KO:K05546] [EC:3.2.1.207]
100953384  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
100954656  CANX; calnexin [KO:K08054]
100944942  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
100942739  CALR; calreticulin [KO:K08057]
100954043  calreticulin-like [KO:K08057]
100960639  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
100943254  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
100940869  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
100964793  MAN1B1; LOW QUALITY PROTEIN: endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
100953531  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
100957622  LMAN1; protein ERGIC-53 [KO:K10080]
100965588  LMAN1L; protein ERGIC-53-like [KO:K10081]
100951228  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
100942864  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
100959079  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
100950583  SEC13; protein SEC13 homolog [KO:K14004]
100962664  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
100964560  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
100959229  SEC23A; protein transport protein Sec23A [KO:K14006]
100948345  SEC23B; protein transport protein Sec23B [KO:K14006]
100950256  SEC24D; protein transport protein Sec24D [KO:K14007]
100944256  SEC24C; protein transport protein Sec24C [KO:K14007]
100942548  SEC24A; protein transport protein Sec24A [KO:K14007]
100950338  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
100957679  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
100946507  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
100963273  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
100961420  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
100947853  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X2 [KO:K10086]
100951848  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
100960478  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
100951023  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
100966026  endoplasmic reticulum resident protein 29 [KO:K09586]
100954814  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
100964156  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
100941281  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
100959856  OS9; protein OS-9 isoform X3 [KO:K10088]
100953984  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
100952614  SSR1; translocon-associated protein subunit alpha [KO:K13249]
100944859  SSR2; translocon-associated protein subunit beta [KO:K13250]
100946221  SSR3; translocon-associated protein subunit gamma [KO:K13251]
100962342  SSR4; translocon-associated protein subunit delta [KO:K04571]
100947118  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
100942731  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
100951822  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
100964192  DERL1; derlin-1 [KO:K11519]
100955303  DERL3; derlin-3 isoform X1 [KO:K13989]
100965626  DERL2; derlin-2 isoform X1 [KO:K13989]
100950221  UBXN4; UBX domain-containing protein 4 [KO:K24348]
100946248  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
100965929  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
100954299  UBXN6; UBX domain-containing protein 6 [KO:K14011]
100941584  LOW QUALITY PROTEIN: UBX domain-containing protein 6-like [KO:K14011]
100952159  NSFL1C; NSFL1 cofactor p47 isoform X2 [KO:K14012]
100948453  SVIP; small VCP/p97-interacting protein [KO:K14014]
100951930  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
100941958  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
100957591  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
100945458  heat shock 70 kDa protein 1 [KO:K03283]
100959513  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
100956095  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
100945147  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
100946722  heat shock 70 kDa protein 1 [KO:K03283]
100949074  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
100941208  LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 1-like [KO:K09502]
105887721  LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 1-like [KO:K09502]
100956607  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
100961985  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
100941727  DNAJB2; dnaJ homolog subfamily B member 2 isoform X3 [KO:K09508]
100943717  DNAJB12; dnaJ homolog subfamily B member 12 [KO:K09518]
100960713  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
100959322  DNAJC5G; LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 5G [KO:K09525]
100955535  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
100954869  LOW QUALITY PROTEIN: heat shock protein HSP 90-alpha-like [KO:K04079]
100959852  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
100943365  HSPH1; LOW QUALITY PROTEIN: heat shock protein 105 kDa [KO:K09485]
100946538  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
100948464  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
100964243  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
100961607  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
100949170  CRYAA; alpha-crystallin A chain [KO:K09541]
100960925  CRYAB; alpha-crystallin B chain [KO:K09542]
100964688  YOD1; ubiquitin thioesterase OTU1 isoform X1 [KO:K13719] [EC:3.1.2.-]
100966836  PLAA; phospholipase A-2-activating protein [KO:K14018]
100943056  RAD23A; UV excision repair protein RAD23 homolog A [KO:K10839]
100940792  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
100942674  UBQLN2; ubiquilin-2 [KO:K04523]
100951268  UBQLN4; ubiquilin-4 isoform X2 [KO:K04523]
100952354  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
100946756  ubiquilin-3 [KO:K04523]
100956422  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
100952072  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
100954410  UBE4B; ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
100945512  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
100966174  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
100962266  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
100940993  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
100947110  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
100956595  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
100955447  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
100945547  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
100951769  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X2 [KO:K09049]
100956334  WFS1; wolframin [KO:K14020]
100958393  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
100966464  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
100960586  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
100942489  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
100966378  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
100943232  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
100966900  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X4 [KO:K04431] [EC:2.7.12.2]
100957169  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
100967053  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
100960275  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
100957285  BAX; apoptosis regulator BAX [KO:K02159]
100941755  BAK1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
100950762  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
100946093  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
100954053  LOW QUALITY PROTEIN: caspase-12-like [KO:K04741] [EC:3.4.22.-]
100948823  MARCH6; LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
100941067  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
100959444  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
100942022  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
100944017  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
100950145  SYVN1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
100944170  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
100955007  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
100966453  SELENOS; selenoprotein S [KO:K14025]
100960356  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
100956790  SEL1L; protein sel-1 homolog 1 [KO:K14026]
100942706  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
100962272  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
100942699  STUB1; E3 ubiquitin-protein ligase CHIP isoform X2 [KO:K09561] [EC:2.3.2.27]
100959697  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
100951899  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
100964707  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X4 [KO:K06689] [EC:2.3.2.23]
100950162  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
100965001  PRKN; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
100963359  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
100962270  CUL1; cullin-1 [KO:K03347]
100947924  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
100959039  FBXO2; F-box only protein 2 [KO:K10099]
100956609  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
oga00510  N-Glycan biosynthesis
oga03050  Proteasome
oga03060  Protein export
oga04210  Apoptosis
oga04930  Type II diabetes mellitus
oga05010  Alzheimer disease
oga05012  Parkinson disease
oga05020  Prion disease
KO pathway
ko04141   
LinkDB

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