KEGG   PATHWAY: mpah04141
Entry
mpah04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Mus pahari (shrew 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
mpah04141  Protein processing in endoplasmic reticulum
mpah04141

Other DBs
GO: 0030433 0034976
Organism
Mus pahari (shrew mouse) [GN:mpah]
Gene
110315724  Sec61a1; protein transport protein Sec61 subunit alpha [KO:K10956]
110333596  Sec61a2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
110323530  Sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
110325321  protein transport protein Sec61 subunit gamma-like [KO:K07342]
110330908  Sec61g; protein transport protein Sec61 subunit gamma isoform X1 [KO:K07342]
110319998  Sec62; translocation protein SEC62 [KO:K12275]
110326386  Sec63; translocation protein SEC63 homolog [KO:K09540]
110314113  Rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 isoform X1 [KO:K12666]
110318051  Rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
110325627  Dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
110322885  Ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
110328703  Stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
110327270  Stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
110313528  Magt1; magnesium transporter protein 1 isoform X1 [KO:K19478]
110336436  Tusc3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
110320356  Ostc; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
110332655  Tmem258; transmembrane protein 258 [KO:K26502]
110316001  Mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
110326417  Ckap4; cytoskeleton-associated protein 4 [KO:K13999]
110318350  Rrbp1; ribosome-binding protein 1 [KO:K14000]
110333136  Sil1; nucleotide exchange factor SIL1 [KO:K14001]
110327579  Hyou1; hypoxia up-regulated protein 1 [KO:K09486]
110319438  Hspa5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
110329364  Dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
110318509  Dnajc1; dnaJ homolog subfamily C member 1 [KO:K09521]
110325430  Dnajc3; dnaJ homolog subfamily C member 3 [KO:K09523]
110317647  Dnajc10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
110326056  Hsp90b1; endoplasmin [KO:K09487]
110337924  Ganab; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
110327507  Prkcsh; glucosidase 2 subunit beta isoform X1 [KO:K08288]
110331220  Canx; calnexin [KO:K08054]
110317720  Pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
110337259  Calr; calreticulin [KO:K08057]
110323460  calreticulin-like isoform X1 [KO:K08057]
110326269  Man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
110323014  Man1c1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
110319841  Man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
110317719  Man1b1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
110333665  Lman2; vesicular integral-membrane protein VIP36 [KO:K10082]
110333023  Lman1; protein ERGIC-53 [KO:K10080]
110328037  Lman1l; protein ERGIC-53-like isoform X1 [KO:K10081]
110324481  Preb; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
110331927  Sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
110326285  Sar1a; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
110316450  Sec13; protein SEC13 homolog [KO:K14004]
110329358  Sec31b; LOW QUALITY PROTEIN: protein transport protein Sec31B [KO:K14005]
110330254  Sec31a; protein transport protein Sec31A [KO:K14005]
110319013  Sec23b; protein transport protein Sec23B isoform X1 [KO:K14006]
110324019  Sec23a; protein transport protein Sec23A [KO:K14006]
110319732  Sec24d; protein transport protein Sec24D [KO:K14007]
110320263  Sec24b; protein transport protein Sec24B [KO:K14007]
110325218  Sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
110331428  Sec24a; protein transport protein Sec24A [KO:K14007]
110325386  Uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
110321197  Uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
110316448  Edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
110317724  Edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
110321237  Edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
110331066  P4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
110315481  Pdia4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
110324967  Pdia6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
110313203  Erp29; endoplasmic reticulum resident protein 29 [KO:K09586]
110333722  Txndc5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
110325336  Ero1a; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
110333812  Ero1b; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
110326345  Os9; protein OS-9 isoform X1 [KO:K10088]
110330549  Erlec1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
110333639  Ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
110320281  Ssr2; translocon-associated protein subunit beta isoform X1 [KO:K13250]
110320093  Ssr3; translocon-associated protein subunit gamma [KO:K13251]
110313529  Ssr4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
110313462  Bcap31; B-cell receptor-associated protein 31 [KO:K14009]
110338700  Tram1; translocating chain-associated membrane protein 1 isoform X1 [KO:K14010]
110320365  Tram1l1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
110334616  Derl1; derlin-1 isoform X1 [KO:K11519]
110331615  Derl2; derlin-2 isoform X1 [KO:K13989]
110326809  Derl3; derlin-3 isoform X1 [KO:K13989]
110321495  Ubxn4; UBX domain-containing protein 4 [KO:K24348]
110325366  Ubxn1; UBX domain-containing protein 1 [KO:K24348]
110324466  Ubxn2a; UBX domain-containing protein 2A [KO:K24349]
110335638  Ubxn6; UBX domain-containing protein 6 [KO:K14011]
110336895  Ubxn8; UBX domain-containing protein 8 [KO:K24351]
110317620  Nsfl1c; NSFL1 cofactor p47 isoform X1 [KO:K14012]
110329198  Svip; small VCP/p97-interacting protein [KO:K14014]
110338774  Vcp; transitional endoplasmic reticulum ATPase [KO:K13525]
110331562  Nploc4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
110329556  Ufd1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
110335695  Hspa1l; heat shock 70 kDa protein 1-like [KO:K03283]
110335998  heat shock 70 kDa protein 1A [KO:K03283]
110327293  Hspa8; heat shock cognate 71 kDa protein [KO:K03283]
110338659  Dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502]
110337255  Dnaja2; dnaJ homolog subfamily A member 2 [KO:K09503]
110337241  Dnajb1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
110321539  Dnajb2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
110326424  Dnajb12; LOW QUALITY PROTEIN: dnaJ homolog subfamily B member 12 [KO:K09518]
110320611  Dnajc5b; dnaJ homolog subfamily C member 5B [KO:K09525]
110318635  Dnajc5; dnaJ homolog subfamily C member 5 [KO:K09525]
110324833  Dnajc5g; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
110324187  Hsp90aa1; heat shock protein HSP 90-alpha [KO:K04079]
110339067  Hsph1; heat shock protein 105 kDa isoform X1 [KO:K09485]
110319890  Hspa4l; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
110338748  Bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
110322342  Bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
110336625  Hspbp1; hsp70-binding protein 1 [KO:K09562]
110338059  Cryaa; alpha-crystallin A chain isoform X1 [KO:K09541]
110327474  Cryab; alpha-crystallin B chain [KO:K09542]
110321891  Yod1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
110323054  Plaa; phospholipase A-2-activating protein isoform X1 [KO:K14018]
110323140  Rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
110337519  Rad23a; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
110313615  Ubqln2; ubiquilin-2 isoform X1 [KO:K04523]
110319891  Ubqln4; ubiquilin-4 isoform X1 [KO:K04523]
110333290  ubiquilin-3 [KO:K04523]
110333757  Ubqln1; ubiquilin-1 isoform X1 [KO:K04523]
110325614  Ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
110324276  Atxn3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
110322777  Ube4b; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
110324429  Eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
110318263  Nfe2l2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
110334651  Atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
110331947  Ppp1r15a; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
110326609  Ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
110321591  Bcl2; apoptosis regulator Bcl-2 [KO:K02161]
110321899  Atf6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
110335526  Atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
110330419  Wfs1; wolframin isoform X1 [KO:K14020]
110337284  Mbtps1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
110313738  Mbtps2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
110330475  Xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
110331346  Ern1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
110318405  Traf2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
110337870  Map3k5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
110336400  Map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
110330449  Mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
110332652  Mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
110325466  Mapk8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
110320030  Bax; apoptosis regulator BAX isoform X1 [KO:K02159]
110338169  Bak1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
110316020  Capn1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
110321506  Capn2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
110327565  caspase-12-like [KO:K04741] [EC:3.4.22.-]
110328810  March6; E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
110338773  Ube2j1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
110323130  Ube2j2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
110331669  Ube2g1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
110326535  Ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
110321321  Syvn1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
110330730  Rnf185; E3 ubiquitin-protein ligase RNF185 isoform X1 [KO:K10666] [EC:2.3.2.27]
110335368  Rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
110328221  Selenos; selenoprotein S [KO:K14025]
110318816  Sel1l2; protein sel-1 homolog 2 [KO:K14026]
110324504  Sel1l; protein sel-1 homolog 1 isoform X1 [KO:K14026]
110337405  Herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
110337329  Amfr; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
110338088  Stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
110316544  ubiquitin-conjugating enzyme E2 D1-like [KO:K06689] [EC:2.3.2.23]
110319790  Ube2d3; LOW QUALITY PROTEIN: ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
110330589  ubiquitin-conjugating enzyme E2 D2B [KO:K06689] [EC:2.3.2.23]
110326564  Ube2d1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
110332894  Ube2d2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
110330224  Ube2d4; LOW QUALITY PROTEIN: ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
110313641  ubiquitin-conjugating enzyme E2 D2-like [KO:K06689] [EC:2.3.2.23]
110314009  ubiquitin-conjugating enzyme E2 D4-like [KO:K06689] [EC:2.3.2.23]
110338080  Prkn; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
110334961  Rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
110316251  Cul1; cullin-1 [KO:K03347]
110331774  Skp1; S-phase kinase-associated protein 1 [KO:K03094]
110323621  Fbxo2; F-box only protein 2 [KO:K10099]
110322838  F-box only protein 6 [KO:K10100]
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
mpah00510  N-Glycan biosynthesis
mpah03050  Proteasome
mpah03060  Protein export
mpah04210  Apoptosis
mpah04930  Type II diabetes mellitus
mpah05010  Alzheimer disease
mpah05012  Parkinson disease
mpah05020  Prion disease
KO pathway
ko04141   
LinkDB

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