KEGG   PATHWAY: mmu04141
Entry
mmu04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Mus musculus (house mouse)
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
mmu04141  Protein processing in endoplasmic reticulum
mmu04141

Other DBs
GO: 0030433 0034976
Organism
Mus musculus (house mouse) [GN:mmu]
Gene
100037258  Dnajc3; DnaJ heat shock protein family (Hsp40) member C3 [KO:K09523]
100041121  Gm10144; predicted pseudogene 10144 [KO:K07342]
100046078  Gm15266; predicted gene 15266 [KO:K07342]
100502926  Gm19457; predicted gene, 19457 [KO:K06689] [EC:2.3.2.23]
101055909  Sec61bl; SEC61 translocon subunit beta like [KO:K09481]
102632470  Gm30534; predicted gene, 30534 [KO:K07342]
102636661  Gm33666; predicted gene, 33666 [KO:K09481]
103963  Rpn1; ribophorin I [KO:K12666]
105245  Txndc5; thioredoxin domain containing 5 [KO:K13984] [EC:5.3.4.1]
107513  Ssr1; signal sequence receptor, alpha [KO:K13249]
108159  Ubxn8; UBX domain protein 8 [KO:K24351]
108687  Edem2; ER degradation enhancer, mannosidase alpha-like 2 [KO:K10085]
108802  Calr4; calreticulin 4 [KO:K08057]
109815  Selenos; selenoprotein S [KO:K14025]
110379  Sec13; SEC13 homolog, nuclear pore and COPII coat complex component [KO:K14004]
110616  Atxn3; ataxin 3 [KO:K11863] [EC:3.4.22.-]
115488348  Gm16261; predicted gene 16261 [KO:K07342]
116891  Derl2; Der1-like domain family, member 2 [KO:K13989]
11911  Atf4; activating transcription factor 4 [KO:K04374]
12017  Bag1; BCL2-associated athanogene 1 [KO:K09555]
12018  Bak1; BCL2-antagonist/killer 1 [KO:K14021]
12028  Bax; BCL2-associated X protein [KO:K02159]
12043  Bcl2; B cell leukemia/lymphoma 2 [KO:K02161]
12282  Hyou1; hypoxia up-regulated 1 [KO:K09486]
12304  Pdia4; protein disulfide isomerase associated 4 [KO:K09582] [EC:5.3.4.1]
12317  Calr; calreticulin [KO:K08057]
12330  Canx; calnexin [KO:K08054]
12333  Capn1; calpain 1 [KO:K01367] [EC:3.4.22.52]
12334  Capn2; calpain 2 [KO:K03853] [EC:3.4.22.53]
12364  Casp12; caspase 12 [KO:K04741] [EC:3.4.22.-]
12915  Atf6b; activating transcription factor 6 beta [KO:K09049]
12954  Cryaa; crystallin, alpha A [KO:K09541]
12955  Cryab; crystallin, alpha B [KO:K09542]
13002  Dnajc5; DnaJ heat shock protein family (Hsp40) member C5 [KO:K09525]
13135  Dad1; defender against cell death 1 [KO:K12668]
13198  Ddit3; DNA-damage inducible transcript 3 [KO:K04452]
13200  Ddost; dolichyl-di-phosphooligosaccharide-protein glycotransferase [KO:K12670]
13418  Dnajc1; DnaJ heat shock protein family (Hsp40) member C1 [KO:K09521]
13665  Eif2s1; eukaryotic translation initiation factor 2, subunit 1 alpha [KO:K03237]
13666  Eif2ak3; eukaryotic translation initiation factor 2 alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
140499  Ube2j2; ubiquitin-conjugating enzyme E2J 2 [KO:K04554] [EC:2.3.2.23]
140740  Sec63; SEC63 homolog, protein translocation regulator [KO:K09540]
14376  Ganab; alpha glucosidase 2 alpha neutral subunit [KO:K05546] [EC:3.2.1.207]
14827  Pdia3; protein disulfide isomerase associated 3 [KO:K08056] [EC:5.3.4.1]
14828  Hspa5; heat shock protein 5 [KO:K09490] [EC:3.6.4.10]
15481  Hspa8; heat shock protein 8 [KO:K03283]
15482  Hspa1l; heat shock protein 1-like [KO:K03283]
15502  Dnaja1; DnaJ heat shock protein family (Hsp40) member A1 [KO:K09502]
15505  Hsph1; heat shock 105kDa/110kDa protein 1 [KO:K09485]
15511  Hspa1b; heat shock protein 1B [KO:K03283]
15516  Hsp90ab1; heat shock protein 90 alpha (cytosolic), class B member 1 [KO:K04079]
15519  Hsp90aa1; heat shock protein 90, alpha (cytosolic), class A member 1 [KO:K04079]
16430  Stt3a; STT3, subunit of the oligosaccharyltransferase complex, homolog A (S. cerevisiae) [KO:K07151] [EC:2.4.99.18]
17155  Man1a; mannosidase 1, alpha [KO:K01230] [EC:3.2.1.113]
17156  Man1a2; mannosidase, alpha, class 1A, member 2 [KO:K01230] [EC:3.2.1.113]
17872  Ppp1r15a; protein phosphatase 1, regulatory subunit 15A [KO:K14019]
18024  Nfe2l2; nuclear factor, erythroid derived 2, like 2 [KO:K05638]
18415  Hspa4l; heat shock protein 4 like [KO:K09485]
18453  P4hb; prolyl 4-hydroxylase, beta polypeptide [KO:K09580] [EC:5.3.4.1]
18786  Plaa; phospholipase A2, activating protein [KO:K14018]
19089  Prkcsh; protein kinase C substrate 80K-H [KO:K08288]
192193  Edem1; ER degradation enhancer, mannosidase alpha-like 1 [KO:K10084]
19358  Rad23a; RAD23 homolog A, nucleotide excision repair protein [KO:K10839]
19359  Rad23b; RAD23 homolog B, nucleotide excision repair protein [KO:K10839]
193670  Rnf185; ring finger protein 185 [KO:K10666] [EC:2.3.2.27]
193740  Hspa1a; heat shock protein 1A [KO:K03283]
20014  Rpn2; ribophorin II [KO:K12667]
20224  Sar1a; secretion associated Ras related GTPase 1A [KO:K07953] [EC:3.6.5.-]
20334  Sec23a; SEC23 homolog A, COPII coat complex component [KO:K14006]
20335  Sec61g; SEC61 translocon subunit gamma [KO:K07342]
20338  Sel1l; sel-1 suppressor of lin-12-like (C. elegans) [KO:K14026]
20832  Ssr4; signal sequence receptor, delta [KO:K04571]
213539  Bag2; BCL2-associated athanogene 2 [KO:K09556]
21402  Skp1; S-phase kinase-associated protein 1 [KO:K03094]
216080  Ube2d1; ubiquitin-conjugating enzyme E2D 1 [KO:K06689] [EC:2.3.2.23]
216197  Ckap4; cytoskeleton-associated protein 4 [KO:K13999]
216440  Os9; amplified in osteosarcoma [KO:K10088]
217365  Nploc4; NPL4 homolog, ubiquitin recognition factor [KO:K14015]
217379  Ubxn2a; UBX domain protein 2A [KO:K24349]
218811  Sec24c; SEC24 homolog C, COPII coat complex component [KO:K14007]
22027  Hsp90b1; heat shock protein 90, beta (Grp94), member 1 [KO:K09487]
22030  Traf2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
22213  Ube2g2; ubiquitin-conjugating enzyme E2G 2 [KO:K04555] [EC:2.3.2.23]
22230  Ufd1; ubiquitin recognition factor in ER-associated degradation 1 [KO:K14016]
223455  Marchf6; membrane associated ring-CH-type finger 6 [KO:K10661] [EC:2.3.2.27]
22393  Wfs1; wolframin ER transmembrane glycoprotein [KO:K14020]
22433  Xbp1; X-box binding protein 1 [KO:K09027]
225896  Ubxn1; UBX domain protein 1 [KO:K24348]
226418  Yod1; YOD1 deubiquitinase [KO:K13719] [EC:3.1.2.-]
226641  Atf6; activating transcription factor 6 [KO:K09054]
227619  Man1b1; mannosidase, alpha, class 1B, member 1 [KO:K23741]
228684  Sel1l2; sel-1 suppressor of lin-12-like 2 (C. elegans) [KO:K14026]
229801  Tram1l1; translocation associated membrane protein 1-like 1 [KO:K14010]
230815  Man1c1; mannosidase, alpha, class 1C, member 1 [KO:K01230] [EC:3.2.1.113]
230904  Fbxo2; F-box protein 2 [KO:K10099]
231098  Dnajc5g; DnaJ heat shock protein family (Hsp40) member C5 gamma [KO:K09525]
235416  Lman1l; lectin, mannose-binding 1 like [KO:K10081]
237009  Ube2dnl1; ubiquitin-conjugating enzyme E2D N-terminal like 1 [KO:K06689] [EC:2.3.2.23]
23802  Amfr; autocrine motility factor receptor [KO:K10636] [EC:2.3.2.36]
240667  Sec31b; SEC31 homolog B, COPII coat complex component [KO:K14005]
244178  Ubqln3; ubiquilin 3 [KO:K04523]
26400  Map2k7; mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
26408  Map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
26414  Mapk10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
26419  Mapk8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
26420  Mapk9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
269523  Vcp; valosin containing protein [KO:K13525]
26965  Cul1; cullin 1 [KO:K03347]
27054  Sec23b; SEC23 homolog B, COPII coat complex component [KO:K14006]
27061  Bcap31; B cell receptor associated protein 31 [KO:K14009]
270669  Mbtps2; membrane-bound transcription factor peptidase, site 2 [KO:K07765] [EC:3.4.24.85]
320011  Uggt1; UDP-glucose glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
386649  Nsfl1c; NSFL1 (p97) cofactor (p47) [KO:K14012]
50527  Ero1a; endoplasmic reticulum oxidoreductase 1 alpha [KO:K10950] [EC:1.8.4.-]
50762  Fbxo6; F-box protein 6 [KO:K10100]
50873  Prkn; parkin RBR E3 ubiquitin protein ligase [KO:K04556] [EC:2.3.2.31]
50907  Preb; prolactin regulatory element binding [KO:K14003]
53421  Sec61a1; SEC61 translocon subunit alpha 1 [KO:K10956]
54197  Rnf5; ring finger protein 5 [KO:K10666] [EC:2.3.2.27]
54609  Ubqln2; ubiquilin 2 [KO:K04523]
56085  Ubqln1; ubiquilin 1 [KO:K04523]
56228  Ube2j1; ubiquitin-conjugating enzyme E2J 1 [KO:K10578] [EC:2.3.2.23]
56424  Stub1; STIP1 homology and U-Box containing protein 1 [KO:K09561] [EC:2.3.2.27]
56438  Rbx1; ring-box 1 [KO:K03868] [EC:2.3.2.32]
56445  Dnaja2; DnaJ heat shock protein family (Hsp40) member A2 [KO:K09503]
56453  Mbtps1; membrane-bound transcription factor peptidase, site 1 [KO:K08653] [EC:3.4.21.112]
56550  Ube2d2a; ubiquitin-conjugating enzyme E2D 2A [KO:K06689] [EC:2.3.2.23]
56709  Dnajb12; DnaJ heat shock protein family (Hsp40) member B12 [KO:K09518]
56812  Dnajb2; DnaJ heat shock protein family (Hsp40) member B2 [KO:K09508]
57377  Mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
57743  Sec61a2; SEC61 translocon subunit alpha 2 [KO:K10956]
59007  Ngly1; N-glycanase 1 [KO:K01456] [EC:3.5.1.52]
63958  Ube4b; ubiquitination factor E4B [KO:K10597] [EC:2.3.2.27]
64209  Herpud1; homocysteine-inducible, endoplasmic reticulum stress-inducible, ubiquitin-like domain member 1 [KO:K14027]
66059  Krtcap2; keratinocyte associated protein 2 [KO:K28202]
66105  Ube2d3; ubiquitin-conjugating enzyme E2D 3 [KO:K06689] [EC:2.3.2.23]
66212  Sec61b; SEC61 translocon subunit beta [KO:K09481]
66245  Hspbp1; HSPA (heat shock 70kDa) binding protein, cytoplasmic cochaperone 1 [KO:K09562]
66256  Ssr2; signal sequence receptor, beta [KO:K13250]
66326  Dnajc5b; DnaJ heat shock protein family (Hsp40) member C5 beta [KO:K09525]
66357  Ostc; oligosaccharyltransferase complex subunit (non-catalytic) [KO:K26501]
66397  Sar1b; secretion associated Ras related GTPase 1B [KO:K07953] [EC:3.6.5.-]
66435  Uggt2; UDP-glucose glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
66530  Ubxn6; UBX domain protein 6 [KO:K14011]
66753  Erlec1; endoplasmic reticulum lectin 1 [KO:K14008]
66861  Dnajc10; DnaJ heat shock protein family (Hsp40) member C10 [KO:K09530] [EC:1.8.4.-]
66890  Lman2; lectin, mannose-binding 2 [KO:K10082]
66967  Edem3; ER degradation enhancer, mannosidase alpha-like 3 [KO:K10086]
67075  Magt1; magnesium transporter 1 [KO:K19478]
67128  Ube2g1; ubiquitin-conjugating enzyme E2G 1 [KO:K10575] [EC:2.3.2.23]
67397  Erp29; endoplasmic reticulum protein 29 [KO:K09586]
67437  Ssr3; signal sequence receptor, gamma [KO:K13251]
67475  Ero1b; endoplasmic reticulum oxidoreductase 1 beta [KO:K10976] [EC:1.8.4.-]
67812  Ubxn4; UBX domain protein 4 [KO:K24348]
67819  Derl1; Der1-like domain family, member 1 [KO:K11519]
67838  Dnajb11; DnaJ heat shock protein family (Hsp40) member B11 [KO:K09517]
68292  Stt3b; STT3, subunit of the oligosaccharyltransferase complex, homolog B (S. cerevisiae) [KO:K07151] [EC:2.4.99.18]
69038  Tmem258; transmembrane protein 258 [KO:K26502]
69162  Sec31a; SEC31 homolog A, COPII coat complex component [KO:K14005]
69276  Sec62; SEC62 homolog, preprotein translocation [KO:K12275]
69608  Sec24d; SEC24 homolog D, COPII coat complex component [KO:K14007]
70361  Lman1; lectin, mannose-binding, 1 [KO:K10080]
70377  Derl3; Der1-like domain family, member 3 [KO:K13989]
71853  Pdia6; protein disulfide isomerase associated 6 [KO:K09584] [EC:5.3.4.1]
72265  Tram1; translocating chain-associating membrane protein 1 [KO:K14010]
73318  Ube2d2b; ubiquitin-conjugating enzyme E2D 2B [KO:K06689] [EC:2.3.2.23]
74126  Syvn1; synovial apoptosis inhibitor 1, synoviolin [KO:K10601] [EC:2.3.2.27]
75097  Ube2dnl2; ubiquitin-conjugating enzyme E2D N-terminal like 2 [KO:K06689] [EC:2.3.2.23]
75744  Svip; small VCP/p97-interacting protein [KO:K14014]
77371  Sec24a; SEC24 homolog A, COPII coat complex component [KO:K14007]
78943  Ern1; endoplasmic reticulum to nucleus signalling 1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
80286  Tusc3; tumor suppressor candidate 3 [KO:K19478]
81489  Dnajb1; DnaJ heat shock protein family (Hsp40) member B1 [KO:K09507]
81500  Sil1; SIL1 nucleotide exchange factor [KO:K14001]
81910  Rrbp1; ribosome binding protein 1 [KO:K14000]
94232  Ubqln4; ubiquilin 4 [KO:K04523]
99683  Sec24b; SEC24 homolog B, COPII coat complex component [KO:K14007]
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
mmu00510  N-Glycan biosynthesis
mmu03050  Proteasome
mmu03060  Protein export
mmu04210  Apoptosis
mmu04930  Type II diabetes mellitus
mmu05010  Alzheimer disease
mmu05012  Parkinson disease
mmu05020  Prion disease
KO pathway
ko04141   

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