KEGG   PATHWAY: ocu04141
Entry
ocu04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Oryctolagus cuniculus (rabbit)
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
ocu04141  Protein processing in endoplasmic reticulum
ocu04141

Other DBs
GO: 0030433 0034976
Organism
Oryctolagus cuniculus (rabbit) [GN:ocu]
Gene
100008764  [KO:K09490] [EC:3.6.4.10]
100009050  CALR; calreticulin precursor [KO:K08057]
100009089  [KO:K01367] [EC:3.4.22.52]
100009092  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
100009182  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
100009294  CRYAA; alpha-crystallin A chain [KO:K09541]
100009295  CRYAB; alpha-crystallin B chain [KO:K09542]
100009456  [KO:K09487]
100009532  SSR1; translocon-associated protein subunit alpha precursor [KO:K13249]
100328595  P4HB; protein disulfide-isomerase precursor [KO:K09580] [EC:5.3.4.1]
100328685  PDIA3; protein disulfide-isomerase A3 precursor [KO:K08056] [EC:5.3.4.1]
100328696  SEC63; translocation protein SEC63 homolog [KO:K09540]
100328744  SSR4; translocon-associated protein subunit delta precursor [KO:K04571]
100328875  UBQLN3; ubiquilin-3 [KO:K04523]
100337742  [KO:K07151] [EC:2.4.99.18]
100337999  [KO:K04440] [EC:2.7.11.24]
100338006  [KO:K14006]
100338160  [KO:K03283]
100338258  [KO:K10956]
100338385  [KO:K05546] [EC:3.2.1.207]
100338439  [KO:K04452]
100338665  [KO:K01230] [EC:3.2.1.113]
100338694  [KO:K04079]
100338900  [KO:K10956]
100339093  [KO:K03237]
100339383  [KO:K04374]
100339463  [KO:K03094]
100339485  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
100339717  [KO:K06689] [EC:2.3.2.23]
100339785  [KO:K13525]
100339940  [KO:K09027]
100340043  [KO:K03094]
100340337  [KO:K14007]
100340384  [KO:K07953] [EC:3.6.5.-]
100340398  [KO:K11718] [EC:2.4.1.-]
100340419  [KO:K13251]
100340498  [KO:K04426] [EC:2.7.11.25]
100340644  [KO:K14007]
100340741  [KO:K14012]
100341176  [KO:K06689] [EC:2.3.2.23]
100341242  [KO:K19478]
100341530  [KO:K10578] [EC:2.3.2.23]
100341645  [KO:K08054]
100341860  [KO:K12666]
100341877  [KO:K04523]
100341880  [KO:K12670]
100341962  [KO:K09521]
100342046  [KO:K14021]
100342185  [KO:K24349]
100342354  [KO:K24349]
100342387  [KO:K13250]
100342464  [KO:K10575] [EC:2.3.2.23]
100342533  [KO:K10636] [EC:2.3.2.36]
100342564  [KO:K14005]
100342664  [KO:K04440] [EC:2.7.11.24]
100343272  [KO:K04555] [EC:2.3.2.23]
100343418  [KO:K07953] [EC:3.6.5.-]
100343826  [KO:K09517]
100344035  [KO:K08653] [EC:3.4.21.112]
100344248  [KO:K14007]
100344417  [KO:K28202]
100344429  [KO:K13719] [EC:3.1.2.-]
100344470  [KO:K14027]
100344858  [KO:K07765] [EC:3.4.24.85]
100344895  [KO:K06689] [EC:2.3.2.23]
100344900  [KO:K10085]
100345469  [KO:K09555]
100345546  [KO:K10086]
100345752  [KO:K03347]
100345788  [KO:K13989]
100345796  [KO:K10088]
100346284  [KO:K07342]
100346313  [KO:K10084]
100346462  [KO:K09556]
100346508  [KO:K09502]
100346580  [KO:K09584] [EC:5.3.4.1]
100346880  [KO:K13249]
100346929  [KO:K04440] [EC:2.7.11.24]
100347089  [KO:K04523]
100347427  SELENOS; selenoprotein S isoform X1 [KO:K14025]
100347511  [KO:K06689] [EC:2.3.2.23]
100347643  [KO:K14001]
100347699  [KO:K10081]
100347726  [KO:K14010]
100347763  [KO:K10976] [EC:1.8.4.-]
100347777  [KO:K06689] [EC:2.3.2.23]
100347967  [KO:K14008]
100348170  [KO:K10950] [EC:1.8.4.-]
100348537  [KO:K07151] [EC:2.4.99.18]
100348639  [KO:K14026]
100348858  [KO:K24348]
100348873  [KO:K03283]
100349132  [KO:K01228] [EC:3.2.1.106]
100349294  [KO:K14004]
100349346  [KO:K11863] [EC:3.4.22.-]
100349869  [KO:K05546] [EC:3.2.1.207]
100349968  [KO:K14018]
100350184  [KO:K26501]
100350316  [KO:K04554] [EC:2.3.2.23]
100350417  [KO:K08057]
100350626  [KO:K13989]
100350952  [KO:K09503]
100350980  [KO:K14003]
100351324  [KO:K24348]
100351389  [KO:K10100]
100351467  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
100351677  [KO:K07151] [EC:2.4.99.18]
100351795  [KO:K04523]
100351843  [KO:K14010]
100351911  [KO:K11718] [EC:2.4.1.-]
100351931  [KO:K01456] [EC:3.5.1.52]
100351983  [KO:K09525]
100352309  [KO:K09525]
100352543  [KO:K09485]
100352829  [KO:K09525]
100352882  [KO:K09481]
100353014  [KO:K05638]
100353213  [KO:K26502]
100353303  [KO:K14026]
100353489  [KO:K10839]
100353534  [KO:K03283]
100353960  [KO:K10575] [EC:2.3.2.23]
100354003  [KO:K09562]
100354026  [KO:K09582] [EC:5.3.4.1]
100354037  [KO:K03283]
100354069  [KO:K06689] [EC:2.3.2.23]
100354112  [KO:K10080]
100354272  [KO:K13999]
100354369  [KO:K01230] [EC:3.2.1.113]
100354435  [KO:K03283]
100354561  [KO:K09586]
100354821  [KO:K26501]
100354847  [KO:K08860] [EC:2.7.11.1]
100354913  [KO:K10601] [EC:2.3.2.27]
100355027  [KO:K11519]
100355120  [KO:K09508]
100355201  [KO:K13984] [EC:5.3.4.1]
100355225  [KO:K06689] [EC:2.3.2.23]
100355363  [KO:K14006]
100355543  [KO:K09523]
100355675  [KO:K02159]
100355816  [KO:K12668]
100356207  [KO:K10597] [EC:2.3.2.27]
100356247  [KO:K14009]
100356525  [KO:K14015]
100356572  [KO:K03283]
100356585  [KO:K10666] [EC:2.3.2.27]
100356592  [KO:K19478]
100357127  [KO:K10082]
100357346  [KO:K08852] [EC:2.7.11.1 3.1.26.-]
100357489  [KO:K09049]
100357513  [KO:K14007]
100357623  [KO:K14019]
100357821  [KO:K14000]
100357982  [KO:K09027]
100358193  [KO:K14005]
100358216  [KO:K09486]
100358287  [KO:K09518]
100358536  [KO:K09054]
100358690  [KO:K04079]
100358908  [KO:K19478]
100358950  [KO:K10666] [EC:2.3.2.27]
100359198  [KO:K24351]
100359231  [KO:K09485]
100359290  [KO:K09530] [EC:1.8.4.-]
100359311  [KO:K12275]
103345313  [KO:K04431] [EC:2.7.12.2]
103346422  [KO:K14020]
103348184  [KO:K12667]
103349397  [KO:K02161]
103349652  [KO:K09555]
103350677  [KO:K04741] [EC:3.4.22.-]
103351004  [KO:K12666]
103351086  [KO:K14014]
103352470  [KO:K03868] [EC:2.3.2.32]
108175354  [KO:K09502]
108175967  [KO:K09507]
108176120  [KO:K06689] [EC:2.3.2.23]
108178685  [KO:K10839]
127486763  [KO:K06689] [EC:2.3.2.23]
127487309  [KO:K19478]
127487751  [KO:K10099]
127488889  [KO:K03283]
127488890  [KO:K03283]
127489051  [KO:K08288]
127489423  [KO:K26501]
127490082  [KO:K09561] [EC:2.3.2.27]
127490365  [KO:K26501]
127490969  [KO:K14012]
127491672  [KO:K09582] [EC:5.3.4.1]
127493063  [KO:K09049]
127493068  [KO:K03283]
Compound
C00076  Calcium cation
G00009  
G00010  
G00011  
G00012  
G10694  
Reference
  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
  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
  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
  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
  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
ocu00510  N-Glycan biosynthesis
ocu03050  Proteasome
ocu03060  Protein export
ocu04210  Apoptosis
ocu04930  Type II diabetes mellitus
ocu05010  Alzheimer disease
ocu05012  Parkinson disease
ocu05020  Prion disease
KO pathway
ko04141   

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