KEGG   PATHWAY: pleu04141
Entry
pleu04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Peromyscus leucopus (white-footed 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
pleu04141  Protein processing in endoplasmic reticulum
pleu04141

Other DBs
GO: 0030433 0034976
Organism
Peromyscus leucopus (white-footed mouse) [GN:pleu]
Gene
114680608  P4hb; LOW QUALITY PROTEIN: protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
114680991  Ufd1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
114680999  Rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
114681028  Sec61a1; protein transport protein Sec61 subunit alpha [KO:K10956]
114681199  Stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
114681519  Canx; calnexin [KO:K08054]
114681520  Mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
114681861  Rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
114681893  Erp29; endoplasmic reticulum resident protein 29 [KO:K09586]
114682080  Syvn1; LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
114682091  Capn1; LOW QUALITY PROTEIN: calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
114682585  Ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
114682757  Sec24b; protein transport protein Sec24B [KO:K14007]
114682798  Ostc; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
114683044  Sec24a; protein transport protein Sec24A [KO:K14007]
114683050  Skp1; S-phase kinase-associated protein 1 [KO:K03094]
114683052  Sar1b; GTP-binding protein SAR1b isoform X1 [KO:K07953] [EC:3.6.5.-]
114683169  Ube2d3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
114683603  Ganab; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
114683630  Ubxn1; LOW QUALITY PROTEIN: UBX domain-containing protein 1 [KO:K24348]
114684002  Stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
114684307  [KO:K14016]
114684418  Capn2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
114684640  Bax; apoptosis regulator BAX isoform X1 [KO:K02159]
114684702  Ppp1r15a; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
114684877  Eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
114685092  Ubxn6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
114685118  Ero1b; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
114685738  Lman1; LOW QUALITY PROTEIN: protein ERGIC-53 [KO:K10080]
114686066  Map2k7; LOW QUALITY PROTEIN: dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
114686218  [KO:K04079]
114686222  Erlec1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
114686328  Derl2; LOW QUALITY PROTEIN: derlin-2 [KO:K13989]
114686852  [KO:K04741] [EC:3.4.22.-]
114686903  Ubqln3; ubiquilin-3 [KO:K04523]
114686934  [KO:K03283]
114687088  Calr; calreticulin [KO:K08057]
114687089  Rad23a; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
114687709  Hspbp1; hsp70-binding protein 1 [KO:K09562]
114687728  [KO:K24349]
114687991  [KO:K04374]
114688223  Dnajb1; dnaJ homolog subfamily B member 1 [KO:K09507]
114688721  Ubqln1; ubiquilin-1 isoform X1 [KO:K04523]
114689239  Lman2; vesicular integral-membrane protein VIP36 [KO:K10082]
114689578  [KO:K06689] [EC:2.3.2.23]
114689580  [KO:K10084]
114689621  Sec13; protein SEC13 homolog [KO:K14004]
114689894  Atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
114690029  Hspa6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
114690085  Dnajc5b; dnaJ homolog subfamily C member 5B isoform X1 [KO:K09525]
114690110  Tram1; translocating chain-associated membrane protein 1 [KO:K14010]
114690253  Cryab; alpha-crystallin B chain [KO:K09542]
114690335  Lman1l; protein ERGIC-53-like [KO:K10081]
114690342  Ssr2; LOW QUALITY PROTEIN: translocon-associated protein subunit beta [KO:K13250]
114690416  Ern1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
114690536  Bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
114690765  [KO:K11718] [EC:2.4.1.-]
114691135  Hspa8; heat shock cognate 71 kDa protein [KO:K03283]
114691173  Hyou1; hypoxia up-regulated protein 1 [KO:K09486]
114691391  Prkn; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
114691448  [KO:K08056] [EC:5.3.4.1]
114691688  [KO:K26501]
114691694  [KO:K04079]
114691760  Sec31a; protein transport protein Sec31A isoform X6 [KO:K14005]
114691777  Mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
114691824  [KO:K06689] [EC:2.3.2.23]
114691983  Edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
114692418  [KO:K07342]
114692474  Uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
114692476  Dnajc3; dnaJ homolog subfamily C member 3 [KO:K09523]
114692555  Man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
114692781  Man1b1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X2 [KO:K23741]
114692795  Traf2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
114693774  Plaa; phospholipase A-2-activating protein [KO:K14018]
114693919  Dnajc1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
114693987  Ube2d4; ubiquitin-conjugating enzyme E2 D4 isoform X2 [KO:K06689] [EC:2.3.2.23]
114693991  Xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
114694031  Rnf185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
114694285  Atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
114694298  Rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
114694418  Mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
114694506  Herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
114694516  Amfr; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
114694827  Derl1; derlin-1 [KO:K11519]
114695150  Tusc3; tumor suppressor candidate 3 [KO:K19478]
114695250  Prkcsh; glucosidase 2 subunit beta isoform X1 [KO:K08288]
114695321  Sec62; translocation protein SEC62 [KO:K12275]
114695429  Ube2j1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
114695481  Selenos; selenoprotein S [KO:K14025]
114695687  Rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
114695777  [KO:K09487]
114695780  Mbtps2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
114696054  Ero1a; ERO1-like protein alpha isoform X1 [KO:K10950] [EC:1.8.4.-]
114696501  Derl3; derlin-3 isoform X1 [KO:K13989]
114696532  Ube2g2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
114696690  Preb; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
114696801  [KO:K06689] [EC:2.3.2.23]
114696939  Ubxn4; UBX domain-containing protein 4 [KO:K24348]
114696970  Yod1; LOW QUALITY PROTEIN: ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
114697115  Dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
114697194  Ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
114697252  Man1c1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
114697289  Ubxn2a; UBX domain-containing protein 2A [KO:K24349]
114697417  Ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
114697429  Dnajc5g; dnaJ homolog subfamily C member 5G isoform X5 [KO:K09525]
114697468  Mbtps1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
114697635  Sel1l; protein sel-1 homolog 1 [KO:K14026]
114697766  Eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
114698005  Cul1; cullin-1 [KO:K03347]
114698497  Ubxn8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
114698542  Ube2d1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
114698565  Man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
114698844  Pdia6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
114699155  Hsph1; heat shock protein 105 kDa isoform X1 [KO:K09485]
114699340  [KO:K03094]
114699348  Mapk8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
114699590  Pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
114700127  Pdia4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
114700203  Sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
114700525  [KO:K14014]
114700616  Hsp90b1; endoplasmin [KO:K09487]
114700818  Nfe2l2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
114701358  Tram1l1; LOW QUALITY PROTEIN: translocating chain-associated membrane protein 1-like 1 [KO:K14010]
114701382  Sec24d; protein transport protein Sec24D isoform X2 [KO:K14007]
114701838  Marchf6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
114702073  Wfs1; wolframin [KO:K14020]
114702342  Sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
114702810  Bcl2; apoptosis regulator Bcl-2 [KO:K02161]
114702849  Ubqln2; ubiquilin-2 [KO:K04523]
114702997  Ckap4; cytoskeleton-associated protein 4 [KO:K13999]
114703084  Hsp90aa1; heat shock protein HSP 90-alpha [KO:K04079]
114703224  [KO:K13525]
114703255  Bag1; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555]
114703260  Dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502]
114703502  Tmem258; transmembrane protein 258 [KO:K26502]
114703590  [KO:K14026]
114703725  Dnajc10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
114704004  Cryaa; alpha-crystallin A chain isoform X2 [KO:K09541]
114704049  [KO:K14006]
114704050  [KO:K14006]
114704162  Dnajc5; dnaJ homolog subfamily C member 5 [KO:K09525]
114704693  Hspa5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
114704724  [KO:K09518]
114704751  Sar1a; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
114704800  Ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
114704810  Os9; protein OS-9 isoform X1 [KO:K10088]
114704882  Magt1; magnesium transporter protein 1 isoform X1 [KO:K19478]
114704974  Stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
114705096  Ube2d2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
114705103  Sil1; nucleotide exchange factor SIL1 [KO:K14001]
114705530  Sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
114705814  Rnf5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
114705847  Atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
114705867  Hspa1a; heat shock 70 kDa protein 1A [KO:K03283]
114705868  [KO:K03283]
114705870  Hspa1l; heat shock 70 kDa protein 1-like [KO:K03283]
114706045  Atxn3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
114706143  Bak1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
114706181  Ssr4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
114706188  Bcap31; B-cell receptor-associated protein 31 [KO:K14009]
114706302  Ssr3; translocon-associated protein subunit gamma [KO:K13251]
114706361  Ube2g1; ubiquitin-conjugating enzyme E2 G1 isoform X1 [KO:K10575] [EC:2.3.2.23]
114706521  [KO:K10085]
114707186  [KO:K03868] [EC:2.3.2.32]
114707623  [KO:K08057]
114707842  Hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
114707915  Krtcap2; keratinocyte-associated protein 2 isoform X1 [KO:K28202]
114708121  Sec31b; protein transport protein Sec31B isoform X1 [KO:K14005]
114708249  Ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
114708440  Sec61a2; protein transport protein Sec61 subunit alpha [KO:K10956]
114708708  Dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
114708885  [KO:K09518]
114709301  Dnajb2; LOW QUALITY PROTEIN: dnaJ homolog subfamily B member 2 [KO:K09508]
114709419  Sec23b; protein transport protein Sec23B [KO:K14006]
114709430  Rrbp1; LOW QUALITY PROTEIN: ribosome-binding protein 1 [KO:K14000]
114709542  Nsfl1c; NSFL1 cofactor p47 isoform X1 [KO:K14012]
114709644  Fbxo2; F-box only protein 2 [KO:K10099]
114709645  Fbxo6; F-box only protein 6 [KO:K10100]
114709666  Ube4b; LOW QUALITY PROTEIN: ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
114709961  Dnaja2; dnaJ homolog subfamily A member 2 [KO:K09503]
114710568  Txndc5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
114710675  Sec63; translocation protein SEC63 homolog [KO:K09540]
114710777  Hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
114711026  Nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
119087818  Sel1l2; LOW QUALITY PROTEIN: protein sel-1 homolog 2 [KO:K14026]
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
pleu00510  N-Glycan biosynthesis
pleu03050  Proteasome
pleu03060  Protein export
pleu04210  Apoptosis
pleu04930  Type II diabetes mellitus
pleu05010  Alzheimer disease
pleu05012  Parkinson disease
pleu05020  Prion disease
KO pathway
ko04141   

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