KEGG   PATHWAY: ccan04141
Entry
ccan04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Castor canadensis (American beaver)
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
ccan04141  Protein processing in endoplasmic reticulum
ccan04141

Other DBs
GO: 0030433 0034976
Organism
Castor canadensis (American beaver) [GN:ccan]
Gene
109674432  [KO:K08852] [EC:2.7.11.1 3.1.26.-]
109674932  Sel1l2; protein sel-1 homolog 2 [KO:K14026]
109675414  [KO:K11718] [EC:2.4.1.-]
109675714  [KO:K04452]
109675874  Hsp90b1; endoplasmin [KO:K09487]
109675902  Sec24a; protein transport protein Sec24A [KO:K14007]
109675942  Cryaa; alpha-crystallin A chain isoform X1 [KO:K09541]
109677038  Capn2; LOW QUALITY PROTEIN: calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
109677727  Pdia6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
109678705  Sec23b; protein transport protein Sec23B [KO:K14006]
109678916  [KO:K13984] [EC:5.3.4.1]
109679787  Preb; LOW QUALITY PROTEIN: prolactin regulatory element-binding protein [KO:K14003]
109679923  Dnajc5g; dnaJ homolog subfamily C member 5G [KO:K09525]
109680107  Tram1l1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
109680402  [KO:K06689] [EC:2.3.2.23]
109681155  Ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
109681282  Calr; calreticulin [KO:K08057]
109681283  Rad23a; UV excision repair protein RAD23 homolog A [KO:K10839]
109681361  Sil1; nucleotide exchange factor SIL1 [KO:K14001]
109681412  [KO:K09502]
109681424  Hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
109681442  Pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
109681451  Hsph1; heat shock protein 105 kDa [KO:K09485]
109681659  Derl1; derlin-1 isoform X1 [KO:K11519]
109681796  Ubxn4; UBX domain-containing protein 4 [KO:K24348]
109682122  Hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
109682318  Ero1b; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
109682353  Atxn3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
109682444  Sec61a2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
109682680  Ppp1r15a; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
109682703  Bax; apoptosis regulator BAX [KO:K02159]
109682965  Ube2d3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
109683046  Man1c1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
109683432  Hspa5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
109683720  Ubxn2a; UBX domain-containing protein 2A [KO:K24349]
109683955  Ero1a; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
109683995  [KO:K14026]
109684269  [KO:K08057]
109684347  Nfe2l2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
109684355  Ufd1l; ubiquitin fusion degradation protein 1 homolog [KO:K14016]
109684449  Bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
109684587  Dnajc5; dnaJ homolog subfamily C member 5 [KO:K09525]
109684702  Sec61a1; protein transport protein Sec61 subunit alpha [KO:K10956]
109685048  Selenos; selenoprotein S [KO:K14025]
109685286  Sar1a; GTP-binding protein SAR1a isoform X1 [KO:K07953] [EC:3.6.5.-]
109685307  Vcp; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
109685361  [KO:K01230] [EC:3.2.1.113]
109685366  [KO:K01230] [EC:3.2.1.113]
109685446  Dnajb12; LOW QUALITY PROTEIN: dnaJ homolog subfamily B member 12 [KO:K09518]
109685727  Tmem258; transmembrane protein 258 [KO:K26502]
109685763  Lman2; vesicular integral-membrane protein VIP36 [KO:K10082]
109685917  Ube2d4; ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
109685941  Sec31b; LOW QUALITY PROTEIN: protein transport protein Sec31B [KO:K14005]
109686231  Ganab; LOW QUALITY PROTEIN: neutral alpha-glucosidase AB [KO:K05546] [EC:3.2.1.207]
109686259  Ube2g1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
109686273  Os9; protein OS-9 isoform X1 [KO:K10088]
109686362  Ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
109686512  Bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
109686692  Bak1; bcl-2 homologous antagonist/killer [KO:K14021]
109686728  Mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
109686737  Canx; calnexin [KO:K08054]
109686738  [KO:K04440] [EC:2.7.11.24]
109686762  [KO:K19478]
109686914  Rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
109686998  Herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
109687155  Capn1; LOW QUALITY PROTEIN: calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
109687157  Syvn1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
109687274  Ostc; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
109687310  Wfs1; wolframin [KO:K14020]
109687625  Dnajc1; LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 1 [KO:K09521]
109687684  [KO:K04523]
109687685  Ubqln1; ubiquilin-1 isoform X1 [KO:K04523]
109687814  Ubxn6; UBX domain-containing protein 6 [KO:K14011]
109687976  Krtcap2; keratinocyte-associated protein 2 [KO:K28202]
109688031  [KO:K04556] [EC:2.3.2.31]
109688260  Tram1; translocating chain-associated membrane protein 1 [KO:K14010]
109688263  [KO:K14010]
109688664  Sec31a; LOW QUALITY PROTEIN: protein transport protein Sec31A [KO:K14005]
109688902  [KO:K19478]
109688960  Sec13; protein SEC13 homolog isoform X1 [KO:K14004]
109689032  [KO:K12275]
109689175  Ube2d2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
109689399  Derl2; derlin-2 isoform X1 [KO:K13989]
109689406  Derl3; derlin-3 [KO:K13989]
109689474  [KO:K11718] [EC:2.4.1.-]
109689481  Skp1; S-phase kinase-associated protein 1 [KO:K03094]
109689505  Mbtps1; LOW QUALITY PROTEIN: membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
109689561  Sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
109689570  [KO:K03283]
109689571  [KO:K03283]
109689572  [KO:K03283]
109689635  Erlec1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
109689643  [KO:K09523]
109689644  Dnajc3; dnaJ homolog subfamily C member 3 [KO:K09523]
109689687  Ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
109689984  [KO:K14006]
109689985  [KO:K14006]
109690244  Xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
109690413  Rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
109690629  Sec63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
109690733  Yod1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
109690915  Lman1; protein ERGIC-53 [KO:K10080]
109691421  Sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
109692165  Ubxn8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
109692213  Ssr1; LOW QUALITY PROTEIN: translocon-associated protein subunit alpha [KO:K13249]
109692218  Txndc5; LOW QUALITY PROTEIN: thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
109692434  Mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
109692582  Prkcsh; glucosidase 2 subunit beta isoform X1 [KO:K08288]
109692834  Ube2j1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
109693025  Atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
109693047  Dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
109693241  Sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
109693342  Hyou1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
109693393  Man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
109693503  [KO:K03283]
109693757  Bcl2; apoptosis regulator Bcl-2 [KO:K02161]
109694053  Cryab; alpha-crystallin B chain [KO:K09542]
109694157  Bcap31; B-cell receptor-associated protein 31 [KO:K14009]
109694191  Mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
109694366  Nsfl1c; NSFL1 cofactor p47 isoform X1 [KO:K14012]
109694730  March6; E3 ubiquitin-protein ligase MARCH6 isoform X1 [KO:K10661] [EC:2.3.2.27]
109694888  Edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
109694910  Stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
109694930  Sec24b; protein transport protein Sec24B isoform X1 [KO:K14007]
109694960  Ube2g2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
109695335  Edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
109695511  Cul1; cullin-1 [KO:K03347]
109695950  Lman1l; protein ERGIC-53-like [KO:K10081]
109696011  Hsp90aa1; heat shock protein HSP 90-alpha [KO:K04079]
109696270  Hspbp1; hsp70-binding protein 1 isoform X1 [KO:K09562]
109696445  Rrbp1; LOW QUALITY PROTEIN: ribosome-binding protein 1 [KO:K14000]
109696483  Rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
109696656  Dnaja2; dnaJ homolog subfamily A member 2 [KO:K09503]
109696873  Ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
109697041  Map3k5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
109697565  Dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
109697613  Atf6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
109697619  Ssr2; translocon-associated protein subunit beta [KO:K13250]
109697620  Ubqln4; ubiquilin-4 [KO:K04523]
109697755  Hspa8; heat shock cognate 71 kDa protein [KO:K03283]
109697788  Fbxo6; F-box only protein 6 [KO:K10100]
109697794  Fbxo2; F-box only protein 2 isoform X1 [KO:K10099]
109697811  Rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
109697824  Ubqln2; ubiquilin-2 [KO:K04523]
109697850  Stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
109697865  Ssr4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
109697944  Ubxn1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
109697987  Man1b1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741]
109698434  Map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
109698694  Plaa; phospholipase A-2-activating protein [KO:K14018]
109698726  Ckap4; cytoskeleton-associated protein 4 [KO:K13999]
109698793  Dnajb1; dnaJ homolog subfamily B member 1 [KO:K09507]
109699010  [KO:K10597] [EC:2.3.2.27]
109699331  Traf2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
109699410  Edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
109699492  Eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
109699662  [KO:K09530] [EC:1.8.4.-]
109699672  Atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
109699788  Rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
109700304  Stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
109700322  Sec24d; protein transport protein Sec24D isoform X1 [KO:K14007]
109700415  Amfr; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
109700633  Erp29; endoplasmic reticulum resident protein 29 [KO:K09586]
109700775  Pdia4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
109701138  Dnajb2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
109701204  Ssr3; translocon-associated protein subunit gamma [KO:K13251]
109701312  Dnajc5b; dnaJ homolog subfamily C member 5B [KO:K09525]
109701421  Ube2d1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
109702287  [KO:K12667]
109702580  Nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
109702591  P4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
109702874  [KO:K06689] [EC:2.3.2.23]
109702904  Sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
109703004  [KO:K04440] [EC:2.7.11.24]
109703120  Svip; small VCP/p97-interacting protein [KO:K14014]
109703327  [KO:K13251]
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
ccan00510  N-Glycan biosynthesis
ccan03050  Proteasome
ccan03060  Protein export
ccan04210  Apoptosis
ccan04930  Type II diabetes mellitus
ccan05010  Alzheimer disease
ccan05012  Parkinson disease
ccan05020  Prion disease
KO pathway
ko04141   

DBGET integrated database retrieval system