KEGG   PATHWAY: mcc04141
Entry
mcc04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Macaca mulatta (rhesus monkey)
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
mcc04141  Protein processing in endoplasmic reticulum
mcc04141

Other DBs
GO: 0030433 0034976
Organism
Macaca mulatta (rhesus monkey) [GN:mcc]
Gene
100423634  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
100426528  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X2 [KO:K09525]
100427731  SEC61A2; protein transport protein Sec61 subunit alpha isoform 2 [KO:K10956]
100689487  TMEM258; transmembrane protein 258 [KO:K26502]
114669856  [KO:K03283]
114672113  [KO:K08056] [EC:5.3.4.1]
114674839  [KO:K11863] [EC:3.4.22.-]
114675515  [KO:K14021]
693687  SEC31A; protein transport protein Sec31A isoform X18 [KO:K14005]
693769  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
693818  SSR1; translocon-associated protein subunit alpha precursor [KO:K13249]
693963  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
694419  SEL1L2; protein sel-1 homolog 2 isoform X1 [KO:K14026]
694667  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
694752  SELENOS; selenoprotein S isoform 1 [KO:K14025]
694810  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
695213  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
695757  DNAJC3; dnaJ homolog subfamily C member 3 precursor [KO:K09523]
695825  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
696540  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
696663  BCAP31; B-cell receptor-associated protein 31 isoform X1 [KO:K14009]
696989  SEC62; translocation protein SEC62 [KO:K12275]
697105  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
697241  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
697266  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
697283  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
697339  SEC13; protein SEC13 homolog [KO:K14004]
697449  LMAN1; protein ERGIC-53 precursor [KO:K10080]
697784  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
698019  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
698440  SEC23B; protein transport protein Sec23B [KO:K14006]
698500  SEC23A; protein transport protein Sec23A [KO:K14006]
698707  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
698863  SEC63; translocation protein SEC63 homolog [KO:K09540]
699316  DERL3; derlin-3 isoform X1 [KO:K13989]
699482  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
699568  HSP90B1; endoplasmin precursor [KO:K09487]
699575  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
699736  MAPK10; mitogen-activated protein kinase 10 isoform 1 [KO:K04440] [EC:2.7.11.24]
699908  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
699972  AMFR; E3 ubiquitin-protein ligase AMFR isoform X2 [KO:K10636] [EC:2.3.2.36]
700408  PREB; prolactin regulatory element-binding protein [KO:K14003]
700462  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
700798  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
700825  SVIP; small VCP/p97-interacting protein [KO:K14014]
700842  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
701100  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
701123  TUSC3; tumor suppressor candidate 3 precursor [KO:K19478]
701491  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
702101  BAG1; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555]
702293  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
702408  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
702779  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
702936  DNAJB2; dnaJ homolog subfamily B member 2 [KO:K09508]
703047  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
703085  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
703193  HSPA5; 78 kDa glucose-regulated protein precursor [KO:K09490] [EC:3.6.4.10]
703210  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
703434  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
703765  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
703803  DERL1; derlin-1 [KO:K11519]
703965  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
704134  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
704151  DNAJC1; dnaJ homolog subfamily C member 1 precursor [KO:K09521]
704794  CANX; calnexin isoform X2 [KO:K08054]
705437  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 precursor [KO:K12666]
705787  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
706148  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
706150  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 precursor [KO:K10085]
706518  SSR3; translocon-associated protein subunit gamma [KO:K13251]
706534  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
706781  PDIA6; protein disulfide-isomerase A6 precursor [KO:K09584] [EC:5.3.4.1]
706918  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
707407  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
707521  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
707606  NFE2L2; nuclear factor erythroid 2-related factor 2 [KO:K05638]
707715  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
707831  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X3 [KO:K06689] [EC:2.3.2.23]
707876  PLAA; phospholipase A-2-activating protein [KO:K14018]
707967  UBQLN2; ubiquilin-2 [KO:K04523]
707989  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
708329  ERO1A; ERO1-like protein alpha precursor [KO:K10950] [EC:1.8.4.-]
708391  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
708431  HSP90AA1; heat shock protein 90kDa alpha (cytosolic), class A member 1 [KO:K04079]
708651  SEL1L; protein sel-1 homolog 1 [KO:K14026]
708913  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
708971  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
709412  [KO:K03283]
709457  LMAN1L; protein ERGIC-53-like isoform X1 [KO:K10081]
709716  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
709787  SEC61A1; protein transport protein Sec61 subunit alpha isoform 1 [KO:K10956]
710077  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
710098  MAGT1; magnesium transporter protein 1 [KO:K19478]
710143  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
710150  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
710252  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
710333  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
710581  CUL1; cullin-1 [KO:K03347]
710747  CRYAB; alpha-crystallin B chain [KO:K09542]
710772  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
710862  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
710899  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
710912  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
710948  WFS1; wolframin [KO:K14020]
711029  PDIA3; protein disulfide-isomerase A3 precursor [KO:K08056] [EC:5.3.4.1]
711109  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
711115  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
711119  [KO:K11863] [EC:3.4.22.-]
711269  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
711277  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
711328  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
711382  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
711484  ERP29; endoplasmic reticulum resident protein 29 precursor [KO:K09586]
711548  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
711716  UFD1; ubiquitin fusion degradation protein 1 homolog [KO:K14016]
711840  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
712051  DERL2; derlin-2 [KO:K13989]
712091  UBXN4; UBX domain-containing protein 4 [KO:K24348]
712778  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
713348  UBE4B; ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
713525  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
713863  XBP1; X-box-binding protein 1 isoform XBP1(U) [KO:K09027]
713927  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
714475  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
714488  UBQLN3; ubiquilin-3 [KO:K04523]
714609  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA isoform X1 [KO:K01230] [EC:3.2.1.113]
714650  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
714688  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
714702  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
714706  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
714762  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
714901  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
715070  UBXN6; UBX domain-containing protein 6 [KO:K14011]
715555  OS9; protein OS-9 precursor [KO:K10088]
715743  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
715782  MAPK9; mitogen-activated protein kinase 9 isoform X2 [KO:K04440] [EC:2.7.11.24]
716330  MARCH6; E3 ubiquitin-protein ligase MARCH6 isoform X1 [KO:K10661] [EC:2.3.2.27]
716353  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
716460  ERLEC1; endoplasmic reticulum lectin 1 precursor [KO:K14008]
716618  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
716633  [KO:K03283]
716698  SYVN1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
716898  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
716910  CALR; calreticulin precursor [KO:K08057]
717050  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
717056  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
717234  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
717524  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
717751  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
717815  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
717979  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
718340  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
718350  PPP1R15A; protein phosphatase 1 regulatory subunit 15A isoform X1 [KO:K14019]
718351  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
718643  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
718672  GANAB; neutral alpha-glucosidase AB precursor [KO:K05546] [EC:3.2.1.207]
718890  DNAJB1; dnaJ homolog subfamily B member 1 isoform X2 [KO:K09507]
718948  BAX; apoptosis regulator BAX [KO:K02159]
719383  SSR2; translocon-associated protein subunit beta precursor [KO:K13250]
719393  UBQLN4; ubiquilin-4 [KO:K04523]
719686  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
720001  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
720054  HSPA1A; heat shock 70 kDa protein 1A [KO:K03283]
720056  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
720396  [KO:K09586]
720515  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
721528  UBE2G1; ubiquitin-conjugating enzyme E2 G1 isoform X2 [KO:K10575] [EC:2.3.2.23]
721891  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
721956  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
722370  CRYAA; alpha-crystallin A chain [KO:K09541]
722738  FBXO2; F-box only protein 2 [KO:K10099]
722740  FBXO6; F-box only protein 6 [KO:K10100]
723005  LMAN2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
724039  CASP12; caspase-12 [KO:K04741] [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
mcc00510  N-Glycan biosynthesis
mcc03050  Proteasome
mcc03060  Protein export
mcc04210  Apoptosis
mcc04930  Type II diabetes mellitus
mcc05010  Alzheimer disease
mcc05012  Parkinson disease
mcc05020  Prion disease
KO pathway
ko04141   

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