KEGG   PATHWAY: mdo04141
Entry
mdo04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Monodelphis domestica (gray short-tailed opossum)
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
mdo04141  Protein processing in endoplasmic reticulum
mdo04141

Other DBs
GO: 0030433 0034976
Organism
Monodelphis domestica (gray short-tailed opossum) [GN:mdo]
Gene
100009803  HSPA4L; heat shock protein family A (Hsp70) member 4 like [KO:K09485]
100009871  DNAJB12; DnaJ heat shock protein family (Hsp40) member B12 [KO:K09518]
100009956  UBXN1; UBX domain protein 1 [KO:K24348]
100010274  GANAB; glucosidase II alpha subunit [KO:K05546] [EC:3.2.1.207]
100010432  DNAJB2; DnaJ heat shock protein family (Hsp40) member B2 [KO:K09508]
100010573  SEC31A; SEC31 homolog A, COPII coat complex component [KO:K14005]
100010628  SEC31B; SEC31 homolog B, COPII coat complex component [KO:K14005]
100010690  SEC24C; SEC24 homolog C, COPII coat complex component [KO:K14007]
100010762  PRKCSH; protein kinase C substrate 80K-H [KO:K08288]
100010817  OSTC; oligosaccharyltransferase complex non-catalytic subunit [KO:K26501]
100010838  BAG1; BCL2 associated athanogene 1 [KO:K09555]
100010854  MAN1A2; mannosidase alpha class 1A member 2 [KO:K01230] [EC:3.2.1.113]
100011019  MBTPS2; membrane bound transcription factor peptidase, site 2 [KO:K07765] [EC:3.4.24.85]
100011332  SEC61B; Sec61 translocon beta subunit [KO:K09481]
100011342  SAR1A; secretion associated Ras related GTPase 1A [KO:K07953] [EC:3.6.5.-]
100011374  SEC62; SEC62 homolog, preprotein translocation factor [KO:K12275]
100011392  DERL2; derlin 2 [KO:K13989]
100011711  [KO:K03283]
100011727  PDIA3; protein disulfide isomerase family A member 3 [KO:K08056] [EC:5.3.4.1]
100012026  SEC24D; SEC24 homolog D, COPII coat complex component [KO:K14007]
100012165  SSR3; signal sequence receptor subunit 3 [KO:K13251]
100012224  ATF4; activating transcription factor 4 [KO:K04374]
100012279  UBXN4; UBX domain protein 4 [KO:K24348]
100012869  UBE2G2; ubiquitin conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
100012950  DNAJA2; DnaJ heat shock protein family (Hsp40) member A2 [KO:K09503]
100013102  HSP90AB1; heat shock protein 90 alpha family class B member 1 [KO:K04079]
100013261  NSFL1C; NSFL1 cofactor [KO:K14012]
100013348  CUL1; cullin 1 [KO:K03347]
100013430  EDEM1; ER degradation enhancing alpha-mannosidase like protein 1 [KO:K10084]
100013521  RBX1; ring-box 1 [KO:K03868] [EC:2.3.2.32]
100013623  SEC61A2; Sec61 translocon alpha 2 subunit [KO:K10956]
100013744  UBE2D1; ubiquitin conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
100014030  ATXN3; ataxin 3 [KO:K11863] [EC:3.4.22.-]
100014120  PDIA4; protein disulfide isomerase family A member 4 [KO:K09582] [EC:5.3.4.1]
100014313  UBE2G1; ubiquitin conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
100014364  UBE2D3; ubiquitin conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
100014528  SAR1B; secretion associated Ras related GTPase 1B [KO:K07953] [EC:3.6.5.-]
100014565  CRYAA; crystallin alpha A [KO:K09541]
100014810  SKP1; S-phase kinase associated protein 1 [KO:K03094]
100014854  HSP90B1; heat shock protein 90 beta family member 1 [KO:K09487]
100014880  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
100015032  [KO:K03283]
100015225  AMFR; autocrine motility factor receptor [KO:K10636] [EC:2.3.2.36]
100015490  ERO1A; endoplasmic reticulum oxidoreductase 1 alpha [KO:K10950] [EC:1.8.4.-]
100015495  ATF6; activating transcription factor 6 [KO:K09054]
100015941  HSPA5; heat shock protein family A (Hsp70) member 5 [KO:K09490] [EC:3.6.4.10]
100016135  STT3A; STT3A, catalytic subunit of the oligosaccharyltransferase complex [KO:K07151] [EC:2.4.99.18]
100016171  PLAA; phospholipase A2 activating protein [KO:K14018]
100016570  BCL2; BCL2, apoptosis regulator [KO:K02161]
100016895  SEC24A; SEC24 homolog A, COPII coat complex component [KO:K14007]
100016998  STUB1; STIP1 homology and U-box containing protein 1 [KO:K09561] [EC:2.3.2.27]
100017243  TRAF2; TNF receptor associated factor 2 [KO:K03173] [EC:2.3.2.27]
100017290  HSPBP1; HSPA (Hsp70) binding protein 1 [KO:K09562]
100017334  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
100017483  SYVN1; synoviolin 1 [KO:K10601] [EC:2.3.2.27]
100017584  SSR2; signal sequence receptor subunit 2 [KO:K13250]
100017761  HSP90AA1; heat shock protein 90 alpha family class A member 1 [KO:K04079]
100017913  UBE2J2; ubiquitin conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
100018391  [KO:K10100]
100018430  [KO:K10100]
100018491  UBXN6; UBX domain protein 6 [KO:K14011]
100018497  SEC61G; Sec61 translocon gamma subunit [KO:K07342]
100018509  FBXO2; F-box protein 2 [KO:K10099]
100018672  CAPN2; calpain 2 [KO:K03853] [EC:3.4.22.53]
100018820  BAG2; BCL2 associated athanogene 2 [KO:K09556]
100018961  WFS1; wolframin ER transmembrane glycoprotein [KO:K14020]
100019081  MARCH6; membrane associated ring-CH-type finger 6 [KO:K10661] [EC:2.3.2.27]
100019358  [KO:K14015]
100019384  DNAJC5; DnaJ heat shock protein family (Hsp40) member C5 [KO:K09525]
100019508  [KO:K08057]
100019530  MAGT1; magnesium transporter 1 [KO:K19478]
100019766  DNAJC3; DnaJ heat shock protein family (Hsp40) member C3 [KO:K09523]
100019777  UBXN8; UBX domain protein 8 [KO:K24351]
100020316  UBQLN3; ubiquilin 3 [KO:K04523]
100020436  DNAJB1; DnaJ heat shock protein family (Hsp40) member B1 [KO:K09507]
100020437  HSPH1; heat shock protein family H (Hsp110) member 1 [KO:K09485]
100020582  SSR1; signal sequence receptor subunit 1 [KO:K13249]
100020775  VCP; valosin containing protein [KO:K13525]
100020895  SELENOS; selenoprotein S [KO:K14025]
100020917  TRAM1; translocation associated membrane protein 1 [KO:K14010]
100021035  ERO1B; endoplasmic reticulum oxidoreductase 1 beta [KO:K10976] [EC:1.8.4.-]
100021092  HERPUD1; homocysteine inducible ER protein with ubiquitin like domain 1 [KO:K14027]
100021317  RAD23A; RAD23 homolog A, nucleotide excision repair protein [KO:K10839]
100021322  SEL1L; SEL1L ERAD E3 ligase adaptor subunit [KO:K14026]
100021554  SEC63; SEC63 homolog, protein translocation regulator [KO:K09540]
100021674  UBQLN1; ubiquilin 1 [KO:K04523]
100021881  LMAN1; lectin, mannose binding 1 [KO:K10080]
100021910  KRTCAP2; keratinocyte associated protein 2 [KO:K28202]
100022113  RAD23B; RAD23 homolog B, nucleotide excision repair protein [KO:K10839]
100022202  TUSC3; tumor suppressor candidate 3 [KO:K19478]
100022455  DERL3; derlin 3 [KO:K13989]
100022731  MAN1B1; mannosidase alpha class 1B member 1 [KO:K23741]
100022787  UBQLN4; ubiquilin 4 [KO:K04523]
100023094  EIF2S1; eukaryotic translation initiation factor 2 subunit alpha [KO:K03237]
100023116  CKAP4; cytoskeleton associated protein 4 [KO:K13999]
100023506  [KO:K12666]
100023576  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
100023597  HSPA1A; heat shock 70 kDa protein 1 [KO:K03283]
100023598  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
100024090  RNF5; ring finger protein 5 [KO:K10666] [EC:2.3.2.27]
100024412  EDEM3; ER degradation enhancing alpha-mannosidase like protein 3 [KO:K10086]
100024424  SEC13; SEC13 homolog, nuclear pore and COPII coat complex component [KO:K14004]
100024477  UBE4B; ubiquitination factor E4B [KO:K10597] [EC:2.3.2.27]
100024520  SEC23A; Sec23 homolog A, coat complex II component [KO:K14006]
100025398  SIL1; SIL1 nucleotide exchange factor [KO:K14001]
100025595  P4HB; prolyl 4-hydroxylase subunit beta [KO:K09580] [EC:5.3.4.1]
100025603  ATF6B; activating transcription factor 6 beta [KO:K09049]
100025703  UBE2D2; ubiquitin conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
100025732  UBE2J1; ubiquitin conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
100025846  UBE2D4; ubiquitin conjugating enzyme E2 D4 (putative) [KO:K06689] [EC:2.3.2.23]
100025904  DERL1; derlin 1 [KO:K11519]
100025983  PREB; prolactin regulatory element binding [KO:K14003]
100026035  DNAJC1; DnaJ heat shock protein family (Hsp40) member C1 [KO:K09521]
100026371  UGGT1; UDP-glucose glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
100026418  ERN1; endoplasmic reticulum to nucleus signaling 1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
100026448  CRYAB; crystallin alpha B [KO:K09542]
100026605  NFE2L2; nuclear factor, erythroid 2 like 2 [KO:K05638]
100026752  MAP2K7; mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
100026932  DNAJB11; DnaJ heat shock protein family (Hsp40) member B11 [KO:K09517]
100026941  UGGT2; UDP-glucose glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
100027060  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase non-catalytic subunit [KO:K12670]
100027415  CALR; calreticulin [KO:K08057]
100027503  BAK1; BCL2 antagonist/killer 1 [KO:K14021]
100027641  DNAJC10; DnaJ heat shock protein family (Hsp40) member C10 [KO:K09530] [EC:1.8.4.-]
100027722  TXNDC5; thioredoxin domain containing 5 [KO:K13984] [EC:5.3.4.1]
100027900  SEC61A1; Sec61 translocon alpha 1 subunit [KO:K10956]
100028089  MAPK9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
100028151  RPN1; ribophorin I [KO:K12666]
100028218  CANX; calnexin [KO:K08054]
100028506  TMEM258; transmembrane protein 258 [KO:K26502]
100028582  RNF185; ring finger protein 185 [KO:K10666] [EC:2.3.2.27]
100029273  ERP29; endoplasmic reticulum protein 29 [KO:K09586]
100029362  DNAJC5B; DnaJ heat shock protein family (Hsp40) member C5 beta [KO:K09525]
100029434  UFD1L; ubiquitin fusion degradation 1 like (yeast) [KO:K14016]
100029557  EIF2AK3; eukaryotic translation initiation factor 2 alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
100030246  DAD1; defender against cell death 1 [KO:K12668]
100030301  NPLOC4; NPL4 homolog, ubiquitin recognition factor [KO:K14015]
100030405  CAPN1; calpain 1 [KO:K01367] [EC:3.4.22.52]
100030412  BAX; BCL2 associated X, apoptosis regulator [KO:K02159]
100030619  XBP1; X-box binding protein 1 [KO:K09027]
100030633  HSPA8; heat shock protein family A (Hsp70) member 8 [KO:K03283]
100030934  UBXN2A; UBX domain protein 2A [KO:K24349]
100031060  DNAJC5G; DnaJ heat shock protein family (Hsp40) member C5 gamma [KO:K09525]
100031315  HYOU1; hypoxia up-regulated 1 [KO:K09486]
100031443  SEC23B; Sec23 homolog B, coat complex II component [KO:K14006]
100031487  OS9; OS9, endoplasmic reticulum lectin [KO:K10088]
100031527  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
100031666  NGLY1; N-glycanase 1 [KO:K01456] [EC:3.5.1.52]
100031949  PDIA6; protein disulfide isomerase family A member 6 [KO:K09584] [EC:5.3.4.1]
100031965  LMAN2; lectin, mannose binding 2 [KO:K10082]
100032077  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
100032432  EDEM2; ER degradation enhancing alpha-mannosidase like protein 2 [KO:K10085]
100032502  PARK2; parkin RBR E3 ubiquitin protein ligase [KO:K04556] [EC:2.3.2.31]
100032604  [KO:K04741] [EC:3.4.22.-]
100032606  [KO:K04741] [EC:3.4.22.-]
100032797  [KO:K03283]
100032810  RPN2; ribophorin II [KO:K12667]
100033260  RRBP1; ribosome binding protein 1 [KO:K14000]
100033267  SEL1L2; SEL1L2 ERAD E3 ligase adaptor subunit [KO:K14026]
100616747  YOD1; YOD1 deubiquitinase [KO:K13719] [EC:3.1.2.-]
100616850  SEC24B; SEC24 homolog B, COPII coat complex component [KO:K14007]
100617183  MBTPS1; membrane bound transcription factor peptidase, site 1 [KO:K08653] [EC:3.4.21.112]
100617488  MAN1C1; mannosidase alpha class 1C member 1 [KO:K01230] [EC:3.2.1.113]
100618895  MAPK10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
103093985  LMAN1L; lectin, mannose binding 1 like [KO:K10081]
103094574  [KO:K14000]
103096213  SVIP; small VCP interacting protein [KO:K14014]
103100712  [KO:K03283]
107650310  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
554241  SSR4; signal sequence receptor subunit 4 [KO:K04571]
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
mdo00510  N-Glycan biosynthesis
mdo03050  Proteasome
mdo03060  Protein export
mdo04210  Apoptosis
mdo04930  Type II diabetes mellitus
mdo05010  Alzheimer disease
mdo05012  Parkinson disease
mdo05020  Prion disease
KO pathway
ko04141   

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