KEGG   PATHWAY: rno04141
Entry
rno04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Rattus norvegicus (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
rno04141  Protein processing in endoplasmic reticulum
rno04141

Other DBs
GO: 0030433 0034976
Organism
Rattus norvegicus (rat) [GN:rno]
Gene
100359803  Ube2d4l1; ubiquitin-conjugating enzyme E2D 4 like 1 [KO:K06689] [EC:2.3.2.23]
100360412  Dnajc1; dnaJ homolog subfamily C member 1 [KO:K09521]
100362805  Txndc5; thioredoxin domain-containing protein 5 precursor [KO:K13984] [EC:5.3.4.1]
100363048  Tmem258l5; transmembrane protein 258-like [KO:K26502]
100910823  Derl2; derlin-2 [KO:K13989]
108348108  Hspa1b; heat shock 70 kDa protein 1B [KO:K03283]
108348617  Sec61gl2; protein transport protein Sec61 subunit gamma-like [KO:K07342]
108351999  Sec61gl4; protein transport protein Sec61 subunit gamma-like [KO:K07342]
108352097  Sec61gl5; protein transport protein Sec61 subunit gamma-like [KO:K07342]
108353050  Sec61gl3; protein transport protein Sec61 subunit gamma-like [KO:K07342]
114590  Ubqln1; ubiquilin-1 [KO:K04523]
116502  Bak1; bcl-2 homologous antagonist/killer [KO:K14021]
116554  Mapk8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
116598  Pdia4; protein disulfide-isomerase A4 precursor [KO:K09582] [EC:5.3.4.1]
116643  Vcp; transitional endoplasmic reticulum ATPase [KO:K13525]
116645  Plaa; phospholipase A-2-activating protein [KO:K14018]
116666  Lman1; protein ERGIC-53 precursor [KO:K10080]
116967  Magt1; magnesium transporter protein 1 precursor [KO:K19478]
117030  Erp29; endoplasmic reticulum resident protein 29 precursor [KO:K09586]
120094610  [KO:K11519]
120099227  [KO:K06689] [EC:2.3.2.23]
120100624  [KO:K07342]
134478824  [KO:K07342]
140639  Nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
156117  Casp12; caspase-12 [KO:K04741] [EC:3.4.22.-]
171071  Ppp1r15a; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
171129  Uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 precursor [KO:K11718] [EC:2.4.1.-]
171562  Ero1a; ERO1-like protein alpha precursor [KO:K10950] [EC:1.8.4.-]
192235  Hyou1; hypoxia up-regulated protein 1 precursor [KO:K09486]
192275  Dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 isoform 2 [KO:K12668]
192351  Fbxo6; F-box only protein 6 [KO:K10100]
24224  Bcl2; apoptosis regulator Bcl-2 [KO:K02161]
24273  Cryaa; alpha-crystallin A chain isoform 2 [KO:K09541]
24468  Hspa8; heat shock cognate 71 kDa protein [KO:K03283]
24472  Hspa1a; heat shock 70 kDa protein 1A [KO:K03283]
246146  Hspbp1; hsp70-binding protein 1 [KO:K09562]
24887  Bax; apoptosis regulator BAX [KO:K02159]
24963  Hspa1l; heat shock 70 kDa protein 1-like [KO:K03283]
25272  Mapk10; mitogen-activated protein kinase 10 isoform 2 [KO:K04440] [EC:2.7.11.24]
25420  Cryab; alpha-crystallin B chain [KO:K09542]
25506  P4hb; protein disulfide-isomerase precursor [KO:K09580] [EC:5.3.4.1]
25596  Rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 precursor [KO:K12666]
25617  Hspa5; endoplasmic reticulum chaperone BiP precursor [KO:K09490] [EC:3.6.4.10]
286900  Selenos; selenoprotein S [KO:K14025]
286906  Pdia6; protein disulfide-isomerase A6 precursor [KO:K09584] [EC:5.3.4.1]
287155  Stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
287275  Sec24a; protein transport protein Sec24A [KO:K14007]
287276  Sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
287280  Skp1; S-phase kinase-associated protein 1 [KO:K03094]
288444  Hsph1; heat shock protein 105 kDa [KO:K09485]
289085  Edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
289754  Xbp1; X-box-binding protein 1 isoform XBP1(U) [KO:K09027]
289874  Erlec1; endoplasmic reticulum lectin 1 precursor [KO:K14008]
290783  Tusc3; tumor suppressor candidate 3 precursor [KO:K19478]
290802  Ubxn8; UBX domain-containing protein 8 [KO:K24351]
290994  Lman2; vesicular integral-membrane protein VIP36 precursor [KO:K10082]
29144  Canx; calnexin precursor [KO:K08054]
29153  Capn1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
29154  Capn2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
291673  Sil1; nucleotide exchange factor SIL1 precursor [KO:K14001]
293719  Ubxn1; UBX domain-containing protein 1 [KO:K24348]
293721  Ganab; neutral alpha-glucosidase AB isoform 1 [KO:K05546] [EC:3.2.1.207]
293852  Bcap31; B-cell receptor-associated protein 31 [KO:K14009]
294331  Ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
29435  Ssr4; translocon-associated protein subunit delta precursor [KO:K04571]
294410  Man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
294513  Dnajb12; dnaJ homolog subfamily B member 12 [KO:K09518]
29467  Ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
29468  Pdia3; protein disulfide-isomerase A3 precursor [KO:K08056] [EC:5.3.4.1]
294862  Marchf6; E3 ubiquitin-protein ligase MARCHF6 [KO:K10661] [EC:2.3.2.27]
294912  Sec62; translocation protein SEC62 [KO:K12275]
294993  Hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
295235  Ssr2; translocon-associated protein subunit beta precursor [KO:K13250]
295243  Krtcap2; keratinocyte-associated protein 2 [KO:K28202]
295319  Man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
295461  Sec24b; protein transport protein Sec24B [KO:K14007]
295690  Dnajc10; dnaJ homolog subfamily C member 10 precursor [KO:K09530] [EC:1.8.4.-]
296304  Edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 precursor [KO:K10085]
29702  Eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 precursor [KO:K08860] [EC:2.7.11.1]
297504  Edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
297522  Sec13; protein SEC13 homolog [KO:K14004]
297961  Ube2j1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
297994  Bag1; BAG family molecular chaperone regulator 1 isoform 1L [KO:K09555]
298012  Rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
298068  Sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
298652  Ube4b; ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
298689  Ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
299331  Hsp90aa1; heat shock protein HSP 90-alpha [KO:K04079]
300084  Rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
300445  Prkcsh; glucosidase 2 subunit beta precursor [KO:K08288]
300743  Lman1l; protein ERGIC-53-like precursor [KO:K10081]
301252  Hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
302705  Mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
304766  Ubxn4; UBX domain-containing protein 4 [KO:K24348]
304962  Atf6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
308905  Ubqln3; ubiquilin-3 [KO:K04523]
309433  Sec31b; protein transport protein Sec31B [KO:K14005]
309858  Sec63; translocation protein SEC63 homolog [KO:K09540]
310633  Ubqln4; ubiquilin-4 [KO:K04523]
310843  Sec24d; protein transport protein Sec24D [KO:K14007]
310846  Tram1l1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
311470  Sel1l2; protein sel-1 homolog 2 precursor [KO:K14026]
311483  Rrbp1; ribosome-binding protein 1 [KO:K14000]
311786  Traf2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
312903  Tram1; translocating chain-associated membrane protein 1 [KO:K14010]
313648  Ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit precursor [KO:K12670]
314352  Sel1l; protein sel-1 homolog 1 precursor [KO:K14026]
317396  Ubqln2; ubiquilin-2 [KO:K04523]
360734  Dnajb11; dnaJ homolog subfamily B member 11 precursor [KO:K09517]
360967  Rnf185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
361014  Ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform 1 [KO:K01456] [EC:3.5.1.52]
361091  Uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
361233  Ssr1; translocon-associated protein subunit alpha precursor [KO:K13249]
361273  Sec61a2; protein transport protein Sec61 subunit alpha [KO:K10956]
361367  Amfr; autocrine motility factor receptor [KO:K10636] [EC:2.3.2.36]
361381  Rad23a; UV excision repair protein RAD23 homolog A isoform 1 [KO:K10839]
361384  Dnajb1; dnaJ homolog subfamily B member 1 [KO:K09507]
361712  Syvn1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
361831  Ube2d1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
361842  Sar1a; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
362040  Ostc; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
362226  Sec23b; protein transport protein Sec23B [KO:K14006]
362356  Cul1; cullin-1 [KO:K03347]
362625  Man1c1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
362859  Ckap4; cytoskeleton-associated protein 4 [KO:K13999]
362862  Hsp90b1; endoplasmin precursor [KO:K09487]
362891  Os9; protein OS-9 precursor [KO:K10088]
362912  Derl1; derlin-1 [KO:K11519]
363160  Stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
363332  Ubxn6; UBX domain-containing protein 6 isoform 1 [KO:K14011]
363855  Map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform 1 [KO:K04431] [EC:2.7.12.2]
363982  Yod1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
364755  Ero1b; ERO1-like protein beta isoform 1 [KO:K10976] [EC:1.8.4.-]
365057  Map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
366567  Dnajc5g; dnaJ homolog subfamily C member 5G [KO:K09525]
367149  Bax-ps1; apoptosis regulator BAX-like [KO:K02159]
406169  Atf6b; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
407784  Rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
498013  Ern1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
499157  Svip; small VCP/p97-interacting protein [KO:K14014]
499317  Tmem258; transmembrane protein 258 [KO:K26502]
499579  Dnajc5b; dnaJ homolog subfamily C member 5B [KO:K09525]
499751  Man1b1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
500972  Stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
502595  Tmem258l1; transmembrane protein 258-like [KO:K26502]
50658  Mapk9; mitogen-activated protein kinase 9 isoform 2 [KO:K04440] [EC:2.7.11.24]
54318  Eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
56816  Prkn; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
58817  Sec23a; protein transport protein Sec23A [KO:K14006]
58842  Preb; prolactin regulatory element-binding protein [KO:K14003]
60331  Atxn3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
63880  Dnajc3; dnaJ homolog subfamily C member 3 precursor [KO:K09523]
641452  Ube2d2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
64202  Calr; calreticulin precursor [KO:K08057]
64631  Ube2g1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
64701  Rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 precursor [KO:K12667]
65028  Dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502]
679583  Tmem258l2; transmembrane protein 258-like [KO:K26502]
680121  Haus8l2; heat shock cognate 71 kDa protein-like [KO:K03283]
680191  Tmem258l4; transmembrane protein 258-like [KO:K26502]
681314  [KO:K02159]
685144  Sec24c; protein transport protein Sec24C [KO:K14007]
686092  Tmem258b; transmembrane protein 258 B [KO:K26502]
689134  Sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
689537  Calr4; calreticulin isoform X1 [KO:K08057]
689593  Dnajb2; dnaJ homolog subfamily B member 2 isoform 3 [KO:K09508]
690038  Bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
690138  Tmem258l3; transmembrane protein 258-like [KO:K26502]
690315  Derl3; derlin-3 [KO:K13989]
78947  Mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
79130  Dnajc5; dnaJ homolog subfamily C member 5 [KO:K09525]
79255  Atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
79435  Ube2d2b; ubiquitin-conjugating enzyme E2 D2B [KO:K06689] [EC:2.3.2.23]
80843  Sec61a1; protein transport protein Sec61 subunit alpha isoform 1 [KO:K10956]
81784  Ssr3; translocon-associated protein subunit gamma [KO:K13251]
81920  Ube2d3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
83619  Nfe2l2; nuclear factor erythroid 2-related factor 2 isoform 2 [KO:K05638]
83725  Wfs1; wolframin [KO:K14020]
83809  Nsfl1c; NSFL1 cofactor p47 [KO:K14012]
84026  Dnaja2; dnaJ homolog subfamily A member 2 [KO:K09503]
84478  Ufd1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
85273  Fbxo2; F-box only protein 2 [KO:K10099]
85430  Herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
89842  Mbtps1; membrane-bound transcription factor site-1 protease precursor [KO:K08653] [EC:3.4.21.112]
93646  Sec31a; protein transport protein Sec31A [KO:K14005]
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
rno00510  N-Glycan biosynthesis
rno03050  Proteasome
rno03060  Protein export
rno04210  Apoptosis
rno04930  Type II diabetes mellitus
rno05010  Alzheimer disease
rno05012  Parkinson disease
rno05020  Prion disease
KO pathway
ko04141   

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