KEGG   PATHWAY: tge04141
Entry
tge04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Theropithecus gelada (gelada)
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
tge04141  Protein processing in endoplasmic reticulum
tge04141

Other DBs
GO: 0030433 0034976
Organism
Theropithecus gelada (gelada) [GN:tge]
Gene
112604595  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
112604718  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
112604785  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
112604892  MOGS; mannosyl-oligosaccharide glucosidase isoform X1 [KO:K01228] [EC:3.2.1.106]
112605027  PDIA6; protein disulfide-isomerase A6 isoform X1 [KO:K09584] [EC:5.3.4.1]
112605118  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
112605622  CAPN1; calpain-1 catalytic subunit isoform X1 [KO:K01367] [EC:3.4.22.52]
112605795  HSPA8; heat shock cognate 71 kDa protein isoform X1 [KO:K03283]
112605828  GANAB; neutral alpha-glucosidase AB [KO:K05546] [EC:3.2.1.207]
112605952  [KO:K04741] [EC:3.4.22.-]
112606129  TMEM258; transmembrane protein 258 isoform X1 [KO:K26502]
112606166  CRYAB; alpha-crystallin B chain isoform X1 [KO:K09542]
112606268  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit isoform X1 [KO:K12670]
112606273  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A isoform X1 [KO:K07151] [EC:2.4.99.18]
112606312  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
112606339  KRTCAP2; keratinocyte-associated protein 2 isoform X1 [KO:K28202]
112606536  UBQLN3; ubiquilin-3 [KO:K04523]
112606713  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
112607043  SVIP; small VCP/p97-interacting protein isoform X1 [KO:K14014]
112607129  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
112607814  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
112607839  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
112607869  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
112607878  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
112607962  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741]
112608018  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
112608124  [KO:K03283]
112608453  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
112608522  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
112608599  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
112608625  SEC61B; protein transport protein Sec61 subunit beta isoform X1 [KO:K09481]
112608643  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
112609094  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
112609113  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
112609472  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
112609776  DERL2; derlin-2 isoform X1 [KO:K13989]
112609822  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
112610233  P4HB; protein disulfide-isomerase isoform X1 [KO:K09580] [EC:5.3.4.1]
112610634  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
112610865  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
112610992  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
112611352  LMAN1; protein ERGIC-53 [KO:K10080]
112611529  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
112612218  PPP1R15A; protein phosphatase 1 regulatory subunit 15A isoform X1 [KO:K14019]
112612236  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
112612242  UBXN6; UBX domain-containing protein 6 [KO:K14011]
112612564  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
112612583  CALR; LOW QUALITY PROTEIN: calreticulin [KO:K08057]
112612584  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
112613256  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
112613495  DNAJB1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
112613606  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
112613760  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
112613782  STUB1; E3 ubiquitin-protein ligase CHIP isoform X1 [KO:K09561] [EC:2.3.2.27]
112613912  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
112614249  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
112614262  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
112615092  BCAP31; B-cell receptor-associated protein 31 isoform X1 [KO:K14009]
112615105  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
112615423  MAGT1; magnesium transporter protein 1 [KO:K19478]
112615605  ATXN3L; ataxin-3-like protein isoform X1 [KO:K11863] [EC:3.4.22.-]
112615897  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
112616239  UBQLN2; ubiquilin-2 isoform X1 [KO:K04523]
112617294  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
112618188  SSR3; translocon-associated protein subunit gamma isoform X1 [KO:K13251]
112618229  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
112618258  SEC62; translocation protein SEC62 [KO:K12275]
112618540  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
112618585  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
112618766  SEC61A1; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
112619114  CAPN2; calpain-2 catalytic subunit isoform X1 [KO:K03853] [EC:3.4.22.53]
112619591  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
112619660  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
112620052  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
112620234  CUL1; cullin-1 isoform X1 [KO:K03347]
112620337  PDIA4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
112620674  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
112620846  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
112621286  [KO:K09541]
112622249  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
112622264  BAG2; BAG family molecular chaperone regulator 2 isoform X1 [KO:K09556]
112622707  SEC63; translocation protein SEC63 homolog [KO:K09540]
112622796  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
112622824  BAK1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
112623226  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
112623422  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
112623445  TXNDC5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
112623531  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
112623593  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
112623675  HSP90AB1; heat shock protein HSP 90-beta isoform X1 [KO:K04079]
112623731  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
112623761  HSPA1B; heat shock 70 kDa protein 1B isoform X1 [KO:K03283]
112623762  [KO:K03283]
112624235  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
112624442  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
112624604  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
112624632  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
112624711  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
112624852  WFS1; wolframin [KO:K14020]
112624922  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
112625003  [KO:K06689] [EC:2.3.2.23]
112625062  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
112625100  SEC24B; protein transport protein Sec24B [KO:K14007]
112625421  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
112625504  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
112625743  CANX; LOW QUALITY PROTEIN: calnexin [KO:K08054]
112625821  MARCH6; E3 ubiquitin-protein ligase MARCH6 isoform X1 [KO:K10661] [EC:2.3.2.27]
112625884  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
112625885  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
112625996  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
112626075  SKP1; S-phase kinase-associated protein 1 isoform X1 [KO:K03094]
112626176  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
112626202  SSR2; translocon-associated protein subunit beta isoform X1 [KO:K13250]
112626308  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
112626309  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
112626615  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
112626627  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
112626853  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
112627417  [KO:K06689] [EC:2.3.2.23]
112627423  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
112628278  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
112628559  SEC23A; protein transport protein Sec23A isoform X1 [KO:K14006]
112628572  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
112628666  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
112628704  SEL1L; protein sel-1 homolog 1 [KO:K14026]
112629018  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
112629021  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
112629253  SELENOS; selenoprotein S [KO:K14025]
112629263  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
112629733  [KO:K08057]
112629835  DERL1; derlin-1 isoform X1 [KO:K11519]
112629850  TRAM1; translocating chain-associated membrane protein 1 isoform X1 [KO:K14010]
112629965  [KO:K11863] [EC:3.4.22.-]
112630064  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
112630234  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
112630394  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
112630960  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
112630966  SEC31B; protein transport protein Sec31B [KO:K14005]
112631233  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
112631631  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
112631636  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
112631776  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
112631860  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
112631904  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
112632046  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
112632534  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
112632664  ATF4; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374]
112632725  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
112632738  DNAJC5; dnaJ homolog subfamily C member 5 isoform X1 [KO:K09525]
112632740  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
112632743  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
112632813  FBXO2; F-box only protein 2 isoform X1 [KO:K10099]
112632887  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
112632933  SEL1L2; protein sel-1 homolog 2 isoform X1 [KO:K14026]
112632981  RNF185; E3 ubiquitin-protein ligase RNF185 isoform X1 [KO:K10666] [EC:2.3.2.27]
112633031  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
112633475  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
112633503  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X1 [KO:K03868] [EC:2.3.2.32]
112633780  DERL3; derlin-3 isoform X1 [KO:K13989]
112634041  DDIT3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452]
112634054  OS9; protein OS-9 isoform X1 [KO:K10088]
112634154  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
112634398  FBXO6; F-box only protein 6 [KO:K10100]
112634742  HSP90B1; endoplasmin [KO:K09487]
112635565  ERP29; endoplasmic reticulum resident protein 29 isoform X1 [KO:K09586]
112635801  UBXN4; UBX domain-containing protein 4 [KO:K24348]
112635958  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
112636029  [KO:K14003]
112636216  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
112636485  [KO:K03283]
112636618  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
112636620  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
112636731  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
112636955  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
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
tge00510  N-Glycan biosynthesis
tge03050  Proteasome
tge03060  Protein export
tge04210  Apoptosis
tge04930  Type II diabetes mellitus
tge05010  Alzheimer disease
tge05012  Parkinson disease
tge05020  Prion disease
KO pathway
ko04141   

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