KEGG   PATHWAY: anu04141
Entry
anu04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Arvicanthis niloticus (African grass rat)
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
anu04141  Protein processing in endoplasmic reticulum
anu04141

Other DBs
GO: 0030433 0034976
Organism
Arvicanthis niloticus (African grass rat) [GN:anu]
Gene
117693303  Stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
117693416  [KO:K26502]
117693745  Tmem258; transmembrane protein 258 [KO:K26502]
117694347  [KO:K04079]
117694375  Ssr4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
117694397  Bcap31; B-cell receptor-associated protein 31 [KO:K14009]
117694400  Magt1; magnesium transporter protein 1 [KO:K19478]
117694488  [KO:K06689] [EC:2.3.2.23]
117694495  [KO:K06689] [EC:2.3.2.23]
117694501  Mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
117694509  [KO:K06689] [EC:2.3.2.23]
117694556  Ubqln2; ubiquilin-2 [KO:K04523]
117695077  [KO:K04079]
117695284  Sec31b; protein transport protein Sec31B [KO:K14005]
117695322  [KO:K07342]
117695482  [KO:K06689] [EC:2.3.2.23]
117696239  Hsp90b1; endoplasmin [KO:K09487]
117696356  Os9; protein OS-9 isoform X1 [KO:K10088]
117696554  Ckap4; cytoskeleton-associated protein 4 [KO:K13999]
117696642  Ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
117697114  Sel1l; protein sel-1 homolog 1 [KO:K14026]
117697169  Eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
117697233  Atxn3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
117697557  Hsp90aa1; heat shock protein HSP 90-alpha [KO:K04079]
117697775  Map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
117697882  Hsph1; heat shock protein 105 kDa [KO:K09485]
117697921  Erp29; endoplasmic reticulum resident protein 29 [KO:K09586]
117697962  Selenos; selenoprotein S [KO:K14025]
117698627  Ube2j1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
117698657  Tram1; translocating chain-associated membrane protein 1 [KO:K14010]
117698764  [KO:K26502]
117698957  Hspa8; heat shock cognate 71 kDa protein [KO:K03283]
117698975  Lman1l; protein ERGIC-53-like isoform X1 [KO:K10081]
117699072  Cryab; alpha-crystallin B chain [KO:K09542]
117699123  Stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
117699280  Ubxn1; UBX domain-containing protein 1 [KO:K24348]
117699441  Hyou1; hypoxia up-regulated protein 1 [KO:K09486]
117699627  Sec31a; protein transport protein Sec31A isoform X1 [KO:K14005]
117699648  [KO:K04741] [EC:3.4.22.-]
117700262  Prkn; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
117701010  Map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
117701202  Hspbp1; hsp70-binding protein 1 [KO:K09562]
117702707  Rrbp1; ribosome-binding protein 1 isoform X1 [KO:K14000]
117702915  Rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
117703077  Dnajc5; dnaJ homolog subfamily C member 5 [KO:K09525]
117703096  Dnajc10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
117703103  Nfe2l2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
117703116  Sec23b; protein transport protein Sec23B [KO:K14006]
117703576  Pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
117703648  Sel1l2; protein sel-1 homolog 2 isoform X1 [KO:K14026]
117703863  Nsfl1c; NSFL1 cofactor p47 isoform X1 [KO:K14012]
117703982  Hspa5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
117704310  [KO:K10085]
117705037  Mapk8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
117705156  [KO:K07342]
117705300  Dnajc3; dnaJ homolog subfamily C member 3 [KO:K09523]
117705303  Ero1a; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
117705321  Dnajb2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
117705343  Sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
117705366  Dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
117705414  Ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
117705415  Ganab; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
117705732  Ubxn6; UBX domain-containing protein 6 [KO:K14011]
117705832  [KO:K03283]
117706199  Uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
117706286  [KO:K07342]
117706549  Tram1l1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
117706567  Hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
117706643  Sec24d; protein transport protein Sec24D [KO:K14007]
117706650  Ube2d3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
117706667  Man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
117706871  Sec24b; protein transport protein Sec24B isoform X1 [KO:K14007]
117706939  Ubqln4; ubiquilin-4 [KO:K04523]
117706941  Krtcap2; keratinocyte-associated protein 2 isoform X1 [KO:K28202]
117706988  Ssr3; translocon-associated protein subunit gamma [KO:K13251]
117707128  Sec62; translocation protein SEC62 isoform X1 [KO:K12275]
117707586  Dnajc5b; dnaJ homolog subfamily C member 5B [KO:K09525]
117707797  Ostc; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
117707896  Ssr2; translocon-associated protein subunit beta [KO:K13250]
117708260  Sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
117708264  Vcp; transitional endoplasmic reticulum ATPase [KO:K13525]
117708323  Ube4b; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
117708395  Plaa; phospholipase A-2-activating protein [KO:K14018]
117708538  [KO:K10100]
117708544  Man1c1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
117708552  Capn1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
117708576  Rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
117708784  Fbxo2; F-box only protein 2 [KO:K10099]
117708852  [KO:K08057]
117709019  Syvn1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
117709262  Bag1; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555]
117709319  Ube2j2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
117709332  Ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
117709372  Dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502]
117709813  Sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
117709966  Mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
117710110  P4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
117710505  Derl2; derlin-2 isoform X1 [KO:K13989]
117710640  Canx; calnexin [KO:K08054]
117710672  Sec24a; protein transport protein Sec24A isoform X1 [KO:K14007]
117710740  Ube2g1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
117710754  Ern1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
117710775  Skp1; S-phase kinase-associated protein 1 [KO:K03094]
117710801  Man1b1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
117710914  Traf2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
117711416  Nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
117711961  Stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
117712536  [KO:K06689] [EC:2.3.2.23]
117712747  Hspa6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
117712775  Atf6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
117712796  Sec61g; LOW QUALITY PROTEIN: protein transport protein Sec61 subunit gamma [KO:K07342]
117712804  Erlec1; endoplasmic reticulum lectin 1 [KO:K14008]
117712880  Wfs1; wolframin [KO:K14020]
117712939  Xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
117712959  Ube2d4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
117712962  Svip; small VCP/p97-interacting protein [KO:K14014]
117713025  Rnf185; E3 ubiquitin-protein ligase RNF185 isoform X1 [KO:K10666] [EC:2.3.2.27]
117713548  Ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
117713584  Ubqln1; ubiquilin-1 isoform X1 [KO:K04523]
117713620  Lman2; vesicular integral-membrane protein VIP36 [KO:K10082]
117713636  Sec61a2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
117713966  Txndc5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
117714096  Dnajc1; dnaJ homolog subfamily C member 1 [KO:K09521]
117714123  Prkcsh; glucosidase 2 subunit beta isoform X1 [KO:K08288]
117714176  Ero1b; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
117714909  Sec61a1; protein transport protein Sec61 subunit alpha [KO:K10956]
117715128  Rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
117715254  Eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
117715294  Edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
117715310  Sec13; protein SEC13 homolog [KO:K14004]
117715496  Mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
117716077  Yod1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
117716122  Edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
117716192  Ubxn4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
117716348  Bcl2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
117716823  Sec23a; protein transport protein Sec23A [KO:K14006]
117716909  Pdia6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
117716988  Dnajc5g; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
117717007  Preb; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
117717938  Ufd1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
117718029  Dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
117718135  [KO:K07342]
117718575  Derl1; derlin-1 [KO:K11519]
117719097  Rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
117719125  Atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
117719653  Ppp1r15a; protein phosphatase 1 regulatory subunit 15A isoform X1 [KO:K14019]
117719678  Lman1; protein ERGIC-53 [KO:K10080]
117719810  Ube2d2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
117720155  Sil1; nucleotide exchange factor SIL1 [KO:K14001]
117720889  Pdia4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
117720943  Cul1; cullin-1 [KO:K03347]
117721225  Ubxn8; UBX domain-containing protein 8 [KO:K24351]
117721391  Capn2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
117721709  [KO:K04079]
117721987  Tusc3; tumor suppressor candidate 3 [KO:K19478]
117722241  Uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
117722249  Bax; apoptosis regulator BAX isoform X1 [KO:K02159]
117722626  Bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
117722670  Hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
117722852  Ubqln3; ubiquilin-3 [KO:K04523]
117722858  [KO:K07342]
117722954  [KO:K07342]
117722966  Mbtps1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
117723027  Amfr; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
117723051  Rad23a; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
117723082  Calr; calreticulin [KO:K08057]
117723155  Dnaja2; dnaJ homolog subfamily A member 2 [KO:K09503]
117723237  Dnajb1; dnaJ homolog subfamily B member 1 [KO:K09507]
117723261  Herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
117723741  [KO:K07342]
117723775  Marchf6; E3 ubiquitin-protein ligase MARCHF6 [KO:K10661] [EC:2.3.2.27]
117724312  Dnajb12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
117724317  Sec63; translocation protein SEC63 homolog [KO:K09540]
117724403  [KO:K03283]
117724405  [KO:K03283]
117724492  Bak1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
117724546  Derl3; derlin-3 [KO:K13989]
117724555  Man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA isoform X1 [KO:K01230] [EC:3.2.1.113]
117724566  Sar1a; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
117724646  Cryaa; alpha-crystallin A chain isoform X1 [KO:K09541]
117724754  Atf6b; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
117724783  Ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
117724837  Rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
117724950  Hspa1l; heat shock 70 kDa protein 1-like [KO:K03283]
117724951  [KO:K03283]
117724960  Ube2d1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
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
anu00510  N-Glycan biosynthesis
anu03050  Proteasome
anu03060  Protein export
anu04210  Apoptosis
anu04930  Type II diabetes mellitus
anu05010  Alzheimer disease
anu05012  Parkinson disease
anu05020  Prion disease
KO pathway
ko04141   

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