KEGG   PATHWAY: gas04141
Entry
gas04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Gracilinanus agilis (agile gracile opossum)
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
gas04141  Protein processing in endoplasmic reticulum
gas04141

Other DBs
GO: 0030433 0034976
Organism
Gracilinanus agilis (agile gracile opossum) [GN:gas]
Gene
123230685  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
123230704  SEC13; protein SEC13 homolog [KO:K14004]
123230721  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
123230725  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
123230736  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
123230935  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
123231022  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
123231136  [KO:K03283]
123231523  LMAN1; protein ERGIC-53 [KO:K10080]
123231687  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
123232069  PRKCSH; glucosidase 2 subunit beta [KO:K08288]
123232090  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
123232293  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
123232306  [KO:K13525]
123232365  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
123232427  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
123232455  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
123232511  DERL2; derlin-2 isoform X1 [KO:K13989]
123232539  DNAJA1; LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 1 [KO:K09502]
123232948  SEC24C; protein transport protein Sec24C [KO:K14007]
123232959  DNAJB12; dnaJ homolog subfamily B member 12 [KO:K09518]
123233015  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
123233047  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
123233149  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
123233376  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
123233516  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
123233652  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
123233906  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
123234018  SEL1L; protein sel-1 homolog 1 [KO:K14026]
123234270  [KO:K09580] [EC:5.3.4.1]
123234698  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
123234768  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
123234769  [KO:K09502]
123235123  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
123235432  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
123235483  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
123235711  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
123236171  SELENOS; selenoprotein S [KO:K14025]
123236211  [KO:K13249]
123236574  [KO:K09502]
123236708  [KO:K06689] [EC:2.3.2.23]
123236783  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
123236857  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
123236911  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
123237034  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
123237079  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
123237192  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
123237194  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
123237299  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
123237325  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
123237359  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
123237384  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
123237409  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
123237442  EDEM2; LOW QUALITY PROTEIN: ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
123237458  CANX; calnexin isoform X1 [KO:K08054]
123237465  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
123237571  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
123237640  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
123237641  SEC24A; protein transport protein Sec24A [KO:K14007]
123237694  SEC23A; protein transport protein Sec23A isoform X1 [KO:K14006]
123237723  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
123237856  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
123237885  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
123237998  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
123238085  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 [KO:K10661] [EC:2.3.2.27]
123238123  SEC31B; protein transport protein Sec31B [KO:K14005]
123238254  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
123238271  MAPK8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
123238676  UBQLN3; ubiquilin-3 [KO:K04523]
123238710  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
123239005  [KO:K04741] [EC:3.4.22.-]
123239039  CRYAB; alpha-crystallin B chain [KO:K09542]
123239385  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
123239612  CRYAA; alpha-crystallin A chain isoform X1 [KO:K09541]
123239816  HSPBP1; hsp70-binding protein 1 [KO:K09562]
123239905  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
123239995  [KO:K08054]
123240160  DNAJB2; dnaJ homolog subfamily B member 2 [KO:K09508]
123240267  SSR3; translocon-associated protein subunit gamma isoform X1 [KO:K13251]
123240291  UBE4B; ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
123240309  [KO:K10100]
123240350  TXNDC5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
123240376  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
123240588  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
123240642  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
123241002  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
123241023  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
123241124  [KO:K13999]
123241252  DERL1; derlin-1 isoform X1 [KO:K11519]
123241351  [KO:K04741] [EC:3.4.22.-]
123241453  [KO:K14009]
123241994  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
123242110  HYOU1; LOW QUALITY PROTEIN: hypoxia up-regulated protein 1 [KO:K09486]
123242128  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
123242173  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
123242190  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
123242280  UBXN4; UBX domain-containing protein 4 [KO:K24348]
123242411  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
123242511  CALR; calreticulin [KO:K08057]
123242638  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
123242651  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
123242828  SEC62; translocation protein SEC62 isoform X1 [KO:K12275]
123242888  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
123242928  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
123243046  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
123243096  FBXO2; F-box only protein 2 [KO:K10099]
123243615  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
123243961  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
123244111  SSR2; translocon-associated protein subunit beta [KO:K13250]
123244159  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
123244167  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
123244335  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
123244336  CAPN2; calpain-2 catalytic subunit isoform X1 [KO:K03853] [EC:3.4.22.53]
123244436  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
123244808  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
123244998  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
123245130  [KO:K03283]
123245132  [KO:K03283]
123245162  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
123245395  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
123245437  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
123245724  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
123245810  [KO:K08057]
123246093  UBQLN4; ubiquilin-4 [KO:K04523]
123246200  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
123246664  PRKN; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
123247123  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
123247505  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
123247554  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
123247689  SEC63; translocation protein SEC63 homolog [KO:K09540]
123247733  YOD1; LOW QUALITY PROTEIN: ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
123247815  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA isoform X1 [KO:K01230] [EC:3.2.1.113]
123247851  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
123247967  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
123248456  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
123248587  HSP90B1; endoplasmin [KO:K09487]
123248712  PDIA4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
123248853  [KO:K03283]
123248982  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
123248995  DNAJC1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
123249074  UBQLN1; ubiquilin-1 [KO:K04523]
123249349  [KO:K04079]
123249675  CUL1; cullin-1 [KO:K03347]
123250100  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
123250545  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
123250662  OS9; protein OS-9 isoform X1 [KO:K10088]
123250717  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
123250747  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
123250763  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
123251003  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
123251044  UBXN1; UBX domain-containing protein 1 [KO:K24348]
123251081  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
123251123  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
123251179  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
123251193  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
123251360  SYVN1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
123251503  WFS1; wolframin [KO:K14020]
123251561  [KO:K14014]
123251599  TMEM258; transmembrane protein 258 [KO:K26502]
123251702  [KO:K14007]
123251823  TUSC3; tumor suppressor candidate 3 [KO:K19478]
123251960  [KO:K03094]
123252040  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
123252133  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
123252251  SEC24B; protein transport protein Sec24B [KO:K14007]
123253017  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
123253026  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
123253330  MAGT1; magnesium transporter protein 1 [KO:K19478]
123253368  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
123253440  [KO:K06689] [EC:2.3.2.23]
123253444  [KO:K06689] [EC:2.3.2.23]
123253762  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
123254655  [KO:K10575] [EC:2.3.2.23]
123254943  [KO:K09523]
123254995  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
123255191  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
123256240  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
123256386  [KO:K03283]
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
gas00510  N-Glycan biosynthesis
gas03050  Proteasome
gas03060  Protein export
gas04210  Apoptosis
gas04930  Type II diabetes mellitus
gas05010  Alzheimer disease
gas05012  Parkinson disease
gas05020  Prion disease
KO pathway
ko04141   

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