KEGG   PATHWAY: mcf04141
Entry
mcf04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Macaca fascicularis (crab-eating macaque)
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
mcf04141  Protein processing in endoplasmic reticulum
mcf04141

Other DBs
GO: 0030433 0034976
Organism
Macaca fascicularis (crab-eating macaque) [GN:mcf]
Gene
101864826  DNAJA2; DnaJ homolog subfamily A member 2 [KO:K09503]
101864833  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X2 [KO:K10085]
101864872  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
101864904  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
101864982  NSFL1C; NSFL1 cofactor P47 [KO:K14012]
101865016  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
101865233  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
101865246  UBQLN3; ubiquilin-3 [KO:K04523]
101865386  UBXN6; UBX domain-containing protein 6 isoform X2 [KO:K14011]
101865428  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
101865434  PDIA6; protein disulfide-isomerase A6 isoform X1 [KO:K09584] [EC:5.3.4.1]
101865515  DNAJB1; DnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
101866175  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
101866310  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
101866505  DNAJC10; DnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
101866509  OS9; protein OS-9 isoform X1 [KO:K10088]
101866658  DNAJC5B; DnaJ homolog subfamily C member 5B [KO:K09525]
101866865  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
101867010  CUL1; cullin-1 isoform X1 [KO:K03347]
101867031  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
101867098  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741]
101867266  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
101867309  UBE2G1; ubiquitin-conjugating enzyme E2 G1 isoform X1 [KO:K10575] [EC:2.3.2.23]
101867512  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
101925077  CANX; calnexin isoform X1 [KO:K08054]
101925175  TRAF2; TNF receptor-associated factor 2 isoform X4 [KO:K03173] [EC:2.3.2.27]
101925240  [KO:K03283]
101925254  DNAJB2; DnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
101925285  ERP29; endoplasmic reticulum resident protein 29 isoform X1 [KO:K09586]
101925386  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
101925590  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
101925657  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X2 [KO:K04426] [EC:2.7.11.25]
101925800  DNAJA1; DnaJ homolog subfamily A member 1 [KO:K09502]
101925832  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
101925902  NGLY1; peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase isoform X2 [KO:K01456] [EC:3.5.1.52]
101926002  SSR1; translocon-associated protein subunit alpha isoform X2 [KO:K13249]
101926012  HSPA1L; heat shock 70 kDa protein 1-like isoform X1 [KO:K03283]
101926060  SEL1L; protein sel-1 homolog 1 [KO:K14026]
101926380  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
101926487  HSP90B1; endoplasmin [KO:K09487]
101926576  WFS1; wolframin isoform X1 [KO:K14020]
101926663  DERL1; derlin-1 [KO:K11519]
102114804  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
102114907  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
102115138  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
102115353  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
102115645  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X3 [KO:K10601] [EC:2.3.2.27]
102115795  [KO:K04741] [EC:3.4.22.-]
102116178  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
102116193  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X3 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
102116299  SELENOS; selenoprotein S [KO:K14025]
102116631  UBQLN2; ubiquilin-2 [KO:K04523]
102116756  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
102117377  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
102117460  PDIA4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
102117736  ATXN3; ataxin-3 isoform X4 [KO:K11863] [EC:3.4.22.-]
102117878  SAR1B; GTP-binding protein SAR1b isoform X1 [KO:K07953] [EC:3.6.5.-]
102117938  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
102118150  DERL2; derlin-2 isoform X1 [KO:K13989]
102118492  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
102118497  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
102118665  SEC62; translocation protein SEC62 [KO:K12275]
102119194  SEL1L2; protein sel-1 homolog 2 isoform X3 [KO:K14026]
102119298  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
102119440  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
102119627  TMEM258; transmembrane protein 258 [KO:K26502]
102120335  SEC23B; protein transport protein Sec23B [KO:K14006]
102120498  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
102121086  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
102121272  MAPK10; mitogen-activated protein kinase 10 isoform X4 [KO:K04440] [EC:2.7.11.24]
102121336  CALR; calreticulin [KO:K08057]
102121369  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
102121451  [KO:K26501]
102121575  SSR3; translocon-associated protein subunit gamma [KO:K13251]
102121716  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
102121725  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
102121752  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
102122252  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
102122421  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
102122430  [KO:K06689] [EC:2.3.2.23]
102122692  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
102122731  LMAN1; protein ERGIC-53 [KO:K10080]
102122963  FBXO6; F-box only protein 6 [KO:K10100]
102123310  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
102123533  MAGT1; magnesium transporter protein 1 isoform X1 [KO:K19478]
102123550  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
102123718  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
102123807  [KO:K04079]
102124044  SSR4; translocon-associated protein subunit delta [KO:K04571]
102124388  FBXO2; F-box only protein 2 [KO:K10099]
102126339  MAPK8; mitogen-activated protein kinase 8 isoform X2 [KO:K04440] [EC:2.7.11.24]
102126347  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
102126502  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
102126975  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X2 [KO:K04431] [EC:2.7.12.2]
102127422  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
102127546  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
102127939  SSR2; translocon-associated protein subunit beta isoform X1 [KO:K13250]
102128000  STUB1; E3 ubiquitin-protein ligase CHIP isoform X2 [KO:K09561] [EC:2.3.2.27]
102128035  SEC31A; protein transport protein Sec31A isoform X2 [KO:K14005]
102128339  CRYAB; alpha-crystallin B chain isoform X1 [KO:K09542]
102128912  PPP1R15A; protein phosphatase 1 regulatory subunit 15A isoform X1 [KO:K14019]
102129086  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
102129116  KRTCAP2; keratinocyte-associated protein 2 isoform X1 [KO:K28202]
102129446  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
102129494  SEC23A; protein transport protein Sec23A [KO:K14006]
102129527  DNAJB11; DnaJ homolog subfamily B member 11 [KO:K09517]
102129992  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X2 [KO:K10086]
102130004  BAG1; BAG family molecular chaperone regulator 1 isoform X3 [KO:K09555]
102130115  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
102131294  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
102131422  LMAN1L; protein ERGIC-53-like isoform X3 [KO:K10081]
102131576  [KO:K26502]
102131632  DDIT3; DNA damage-inducible transcript 3 protein isoform X2 [KO:K04452]
102131903  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
102132188  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
102132255  BAX; apoptosis regulator BAX isoform X6 [KO:K02159]
102132483  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X1 [KO:K03868] [EC:2.3.2.32]
102133475  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
102133514  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
102133600  TUSC3; tumor suppressor candidate 3 isoform X2 [KO:K19478]
102134204  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
102134370  SIL1; nucleotide exchange factor SIL1 isoform X3 [KO:K14001]
102134446  PRKN; E3 ubiquitin-protein ligase parkin isoform X3 [KO:K04556] [EC:2.3.2.31]
102134558  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
102134639  TRAM1; translocating chain-associated membrane protein 1 isoform X2 [KO:K14010]
102134673  RRBP1; ribosome-binding protein 1 isoform X2 [KO:K14000]
102134770  DNAJC3; DnaJ homolog subfamily C member 3 [KO:K09523]
102134885  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
102135070  DERL3; derlin-3 isoform X1 [KO:K13989]
102135340  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A isoform X2 [KO:K07151] [EC:2.4.99.18]
102135500  HSPH1; heat shock protein 105 kDa isoform X2 [KO:K09485]
102135853  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
102136287  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
102136358  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
102136557  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
102136802  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
102137586  SEC13; protein SEC13 homolog isoform X3 [KO:K14004]
102137962  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
102138064  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
102138212  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
102138823  DNAJB12; DnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
102139849  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
102139916  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
102140026  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
102140351  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
102140439  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
102140595  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
102140645  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
102140650  UBXN4; UBX domain-containing protein 4 isoform X2 [KO:K24348]
102140826  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
102141333  SVIP; small VCP/p97-interacting protein [KO:K14014]
102141714  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
102142357  BAK1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
102142506  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
102142637  SKP1; S-phase kinase-associated protein 1 isoform X2 [KO:K03094]
102142639  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X2 [KO:K06689] [EC:2.3.2.23]
102142803  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
102143012  UBXN2A; UBX domain-containing protein 2A isoform X2 [KO:K24349]
102143176  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
102143389  ATXN3L; ataxin-3-like protein [KO:K11863] [EC:3.4.22.-]
102143930  [KO:K07953] [EC:3.6.5.-]
102144062  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
102144228  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
102144293  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
102144630  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
102145595  DNAJC1; DnaJ homolog subfamily C member 1 [KO:K09521]
102145881  CRYAA; alpha-crystallin A chain [KO:K09541]
102145895  DNAJC5; DnaJ homolog subfamily C member 5 [KO:K09525]
102146299  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
102146944  PDIA3; protein disulfide-isomerase A3 isoform X1 [KO:K08056] [EC:5.3.4.1]
102147278  SEC63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
107127640  [KO:K26501]
123566859  [KO:K04523]
123567498  [KO:K07151] [EC:2.4.99.18]
123572795  [KO:K03283]
135964335  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
135964444  [KO:K09586]
135965277  [KO:K14026]
135965287  [KO:K14026]
135966387  [KO:K19478]
135969055  [KO:K11863] [EC:3.4.22.-]
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
mcf00510  N-Glycan biosynthesis
mcf03050  Proteasome
mcf03060  Protein export
mcf04210  Apoptosis
mcf04930  Type II diabetes mellitus
mcf05010  Alzheimer disease
mcf05012  Parkinson disease
mcf05020  Prion disease
KO pathway
ko04141   

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