KEGG   PATHWAY: hgl04141
Entry
hgl04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Heterocephalus glaber (naked mole-rat)
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
hgl04141  Protein processing in endoplasmic reticulum
hgl04141

Other DBs
GO: 0030433 0034976
Organism
Heterocephalus glaber (naked mole-rat) [GN:hgl]
Gene
101711449  Sec61a1; Sec61 translocon alpha 1 subunit [KO:K10956]
101724032  Sec61a2; Sec61 translocon alpha 2 subunit [KO:K10956]
101717405  Sec61b; Sec61 translocon beta subunit [KO:K09481]
101724375  Sec61g; Sec61 translocon gamma subunit [KO:K07342]
101722582  Sec62; SEC62 homolog, preprotein translocation factor [KO:K12275]
101701880  Sec63; SEC63 homolog, protein translocation regulator [KO:K09540]
101703063  Rpn1; ribophorin I [KO:K12666]
101706732  Rpn2; ribophorin II [KO:K12667]
101706224  Dad1; defender against cell death 1 [KO:K12668]
101716383  Ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase non-catalytic subunit [KO:K12670]
101700446  Stt3a; STT3A, catalytic subunit of the oligosaccharyltransferase complex [KO:K07151] [EC:2.4.99.18]
101708251  Stt3b; STT3B, catalytic subunit of the oligosaccharyltransferase complex [KO:K07151] [EC:2.4.99.18]
101705381  Magt1; magnesium transporter 1 [KO:K19478]
101697973  Tusc3; tumor suppressor candidate 3 [KO:K19478]
101710061  Ostc; oligosaccharyltransferase complex non-catalytic subunit [KO:K26501]
101698389  Tmem258; transmembrane protein 258 [KO:K26502]
101699668  Mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
101700263  Ckap4; cytoskeleton associated protein 4 [KO:K13999]
101712445  Rrbp1; ribosome binding protein 1 [KO:K14000]
101716450  Sil1; SIL1 nucleotide exchange factor [KO:K14001]
101698405  Hyou1; hypoxia up-regulated 1 [KO:K09486]
101713339  Hspa5; heat shock protein family A (Hsp70) member 5 [KO:K09490] [EC:3.6.4.10]
101724347  Dnajb11; DnaJ heat shock protein family (Hsp40) member B11 [KO:K09517]
101708839  Dnajc1; DnaJ heat shock protein family (Hsp40) member C1 [KO:K09521]
101710818  Dnajc3; DnaJ heat shock protein family (Hsp40) member C3 [KO:K09523]
101714043  Dnajc10; DnaJ heat shock protein family (Hsp40) member C10 [KO:K09530] [EC:1.8.4.-]
101704175  Hsp90b1; heat shock protein 90 beta family member 1 [KO:K09487]
101720326  Ganab; glucosidase II alpha subunit [KO:K05546] [EC:3.2.1.207]
101712131  Prkcsh; protein kinase C substrate 80K-H [KO:K08288]
101711836  Canx; calnexin [KO:K08054]
101721675  Pdia3; protein disulfide isomerase family A member 3 [KO:K08056] [EC:5.3.4.1]
101708651  calreticulin-like [KO:K08057]
101719263  Calr; calreticulin [KO:K08057]
101714110  Man1a1; mannosidase alpha class 1A member 1 [KO:K01230] [EC:3.2.1.113]
101699709  Man1c1; mannosidase alpha class 1C member 1 [KO:K01230] [EC:3.2.1.113]
101700934  Man1a2; mannosidase alpha class 1A member 2 [KO:K01230] [EC:3.2.1.113]
101724891  Man1b1; mannosidase alpha class 1B member 1 [KO:K23741] [EC:3.2.1.209]
101724120  Lman2; lectin, mannose binding 2 [KO:K10082]
101723407  Lman1; lectin, mannose binding 1 [KO:K10080]
101712747  Lman1l; lectin, mannose binding 1 like [KO:K10081]
101707413  Preb; prolactin regulatory element binding [KO:K14003]
101701175  Sar1b; secretion associated Ras related GTPase 1B [KO:K07953] [EC:3.6.5.-]
101713219  Sar1a; secretion associated Ras related GTPase 1A [KO:K07953] [EC:3.6.5.-]
101709886  Sec13; SEC13 homolog, nuclear pore and COPII coat complex component [KO:K14004]
101719009  Sec31a; SEC31 homolog A, COPII coat complex component [KO:K14005]
101719091  Sec23b; Sec23 homolog B, coat complex II component [KO:K14006]
101714949  Sec23a; Sec23 homolog A, coat complex II component [KO:K14006]
101700331  Sec24a; SEC24 homolog A, COPII coat complex component [KO:K14007]
101710672  Sec24c; SEC24 homolog C, COPII coat complex component [KO:K14007]
101706467  Sec24b; SEC24 homolog B, COPII coat complex component [KO:K14007]
101709266  Sec24d; SEC24 homolog D, COPII coat complex component [KO:K14007]
101718371  Uggt1; UDP-glucose glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
101711166  UDP-glucose:glycoprotein glucosyltransferase 1-like [KO:K11718] [EC:2.4.1.-]
101718003  Uggt2; UDP-glucose glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
101709291  Edem1; ER degradation enhancing alpha-mannosidase like protein 1 [KO:K10084]
101700166  Edem2; ER degradation enhancing alpha-mannosidase like protein 2 [KO:K10085]
101710654  Edem3; ER degradation enhancing alpha-mannosidase like protein 3 [KO:K10086]
101712215  P4hb; prolyl 4-hydroxylase subunit beta [KO:K09580] [EC:5.3.4.1]
101702370  Pdia4; protein disulfide isomerase family A member 4 [KO:K09582] [EC:5.3.4.1]
101698236  Pdia6; protein disulfide isomerase family A member 6 [KO:K09584] [EC:5.3.4.1]
101702986  Erp29; endoplasmic reticulum protein 29 [KO:K09586]
101716947  Txndc5; thioredoxin domain containing 5 [KO:K13984] [EC:5.3.4.1]
101716653  Ero1a; endoplasmic reticulum oxidoreductase 1 alpha [KO:K10950] [EC:1.8.4.-]
101723382  Ero1b; endoplasmic reticulum oxidoreductase 1 beta [KO:K10976] [EC:1.8.4.-]
101706809  Os9; OS9, endoplasmic reticulum lectin [KO:K10088]
101717524  Erlec1; endoplasmic reticulum lectin 1 [KO:K14008]
101711029  Ssr1; signal sequence receptor subunit 1 [KO:K13249]
101702420  Ssr2; signal sequence receptor subunit 2 [KO:K13250]
101721516  Ssr3; signal sequence receptor subunit 3 [KO:K13251]
101721283  Ssr4; signal sequence receptor subunit 4 [KO:K04571]
101719761  Bcap31; B-cell receptor-associated protein 31 [KO:K14009]
101699293  Tram1; translocation associated membrane protein 1 [KO:K14010]
101708202  Tram1l1; translocation associated membrane protein 1-like 1 [KO:K14010]
101722706  Derl1; derlin 1 [KO:K11519]
101698064  Derl3; derlin 3 [KO:K13989]
101699036  Derl2; derlin 2 [KO:K13989]
101697764  Ubxn4; UBX domain protein 4 [KO:K24348]
101724463  Ubxn1; UBX domain protein 1 [KO:K24348]
101697965  Ubxn6; UBX domain protein 6 [KO:K14011]
101726065  Ubxn8; UBX domain protein 8 [KO:K24351]
101704155  Nsfl1c; NSFL1 cofactor [KO:K14012]
101699221  Svip; small VCP interacting protein [KO:K14014]
101712176  Vcp; valosin containing protein [KO:K13525]
101704760  Nploc4; NPL4 homolog, ubiquitin recognition factor [KO:K14015]
101714351  Ufd1l; ubiquitin fusion degradation 1 like (yeast) [KO:K14016]
101699805  Hspa1l; heat shock 70 kDa protein 1-like [KO:K03283]
101700637  heat shock 70 kDa protein 1B [KO:K03283]
101701238  Hspa6; heat shock protein family A (Hsp70) member 6 [KO:K03283]
101708313  Hspa8; heat shock protein family A (Hsp70) member 8 [KO:K03283]
101720228  Dnaja1; DnaJ heat shock protein family (Hsp40) member A1 [KO:K09502]
101723341  dnaJ homolog subfamily A member 1 [KO:K09502]
101699482  Dnaja2; DnaJ heat shock protein family (Hsp40) member A2 [KO:K09503]
101715469  Dnajb1; DnaJ heat shock protein family (Hsp40) member B1 [KO:K09507]
101717959  Dnajb2; DnaJ heat shock protein family (Hsp40) member B2 [KO:K09508]
101698082  Dnajb12; DnaJ heat shock protein family (Hsp40) member B12 [KO:K09518]
101718761  Dnajc5b; DnaJ heat shock protein family (Hsp40) member C5 beta [KO:K09525]
101700492  Dnajc5; DnaJ heat shock protein family (Hsp40) member C5 [KO:K09525]
101711964  Dnajc5g; DnaJ heat shock protein family (Hsp40) member C5 gamma [KO:K09525]
101725326  Hsp90aa1; heat shock protein 90 alpha family class A member 1 [KO:K04079]
101710837  heat shock protein HSP 90-alpha-like [KO:K04079]
101726120  heat shock protein HSP 90-beta-like [KO:K04079]
101699803  Hsp90ab1; heat shock protein 90 alpha family class B member 1 [KO:K04079]
101699137  Hspa4l; heat shock protein family A (Hsp70) member 4 like [KO:K09485]
101702785  Hsph1; heat shock protein family H (Hsp110) member 1 [KO:K09485]
101710733  Bag1; BCL2 associated athanogene 1 [KO:K09555]
101718309  Bag2; BCL2 associated athanogene 2 [KO:K09556]
101708996  Hspbp1; HSPA (Hsp70) binding protein 1 [KO:K09562]
101702377  Cryaa; crystallin alpha A [KO:K09541]
101696917  Cryab; crystallin alpha B [KO:K09542]
101721678  Yod1; YOD1 deubiquitinase [KO:K13719] [EC:3.1.2.-]
101708135  Plaa; phospholipase A2 activating protein [KO:K14018]
101696493  Rad23b; RAD23 homolog B, nucleotide excision repair protein [KO:K10839]
101719617  Rad23a; RAD23 homolog A, nucleotide excision repair protein [KO:K10839]
101705561  Ubqln2; ubiquilin 2 [KO:K04523]
101721328  Ubqln3; ubiquilin-3 [KO:K04523]
101715174  Ubqln1; ubiquilin 1 [KO:K04523]
101702766  Ubqln4; ubiquilin 4 [KO:K04523]
101696317  Ngly1; N-glycanase 1 [KO:K01456] [EC:3.5.1.52]
101725084  Atxn3; ataxin 3 [KO:K11863] [EC:3.4.22.-]
101708165  Ube4b; ubiquitination factor E4B [KO:K10597] [EC:2.3.2.27]
101713561  Eif2ak3; eukaryotic translation initiation factor 2 alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
101723170  Eif2s1; eukaryotic translation initiation factor 2 subunit alpha [KO:K03237]
101698153  Nfe2l2; nuclear factor, erythroid 2 like 2 [KO:K05638]
101724287  Atf4; activating transcription factor 4 [KO:K04374]
101715557  Ppp1r15a; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
101715873  Ddit3; DNA damage inducible transcript 3 [KO:K04452]
101701667  Bcl2; BCL2, apoptosis regulator [KO:K02161]
101706597  Atf6; activating transcription factor 6 [KO:K09054]
101715793  Atf6b; activating transcription factor 6 beta [KO:K09049]
101711151  Wfs1; wolframin ER transmembrane glycoprotein [KO:K14020]
101713902  Mbtps1; membrane bound transcription factor peptidase, site 1 [KO:K08653] [EC:3.4.21.112]
101726620  Mbtps2; membrane bound transcription factor peptidase, site 2 [KO:K07765] [EC:3.4.24.85]
101723083  Xbp1; X-box binding protein 1 [KO:K09027]
101714834  uncharacterized LOC101714834 [KO:K09027]
101714734  Ern1; endoplasmic reticulum to nucleus signaling 1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
101705156  Traf2; TNF receptor associated factor 2 [KO:K03173] [EC:2.3.2.27]
101712051  Map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
101710666  Map2k7; mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
101698286  Mapk9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
101715259  Mapk8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
101712980  Mapk10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
101713575  Bax; BCL2 associated X, apoptosis regulator [KO:K02159]
101707336  Bak1; BCL2 antagonist/killer 1 [KO:K14021]
101696511  Capn1; calpain 1 [KO:K01367] [EC:3.4.22.52]
101720177  Capn2; calpain 2 [KO:K03853] [EC:3.4.22.53]
101725565  caspase-12 [KO:K04741] [EC:3.4.22.-]
101724762  March6; membrane associated ring-CH-type finger 6 [KO:K10661] [EC:2.3.2.27]
101723415  Ube2j1; ubiquitin conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
101720519  Ube2j2; ubiquitin conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
101698410  Ube2g1; ubiquitin conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
101701333  Ube2g2; ubiquitin conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
101697993  Syvn1; synoviolin 1 [KO:K10601] [EC:2.3.2.27]
101697701  Rnf185; ring finger protein 185 [KO:K10666] [EC:2.3.2.27]
101708759  Rnf5; ring finger protein 5 [KO:K10666] [EC:2.3.2.27]
101699120  Selenos; selenoprotein S [KO:K14025]
101724591  Sel1l; SEL1L ERAD E3 ligase adaptor subunit [KO:K14026]
101700850  Sel1l2; SEL1L2 ERAD E3 ligase adaptor subunit [KO:K14026]
101703450  Herpud1; homocysteine inducible ER protein with ubiquitin like domain 1 [KO:K14027]
101722534  Amfr; autocrine motility factor receptor [KO:K10636] [EC:2.3.2.36]
101703625  Stub1; STIP1 homology and U-box containing protein 1 [KO:K09561] [EC:2.3.2.27]
106008085  E3 ubiquitin-protein ligase CHIP-like [KO:K09561] [EC:2.3.2.27]
106008736  E3 ubiquitin-protein ligase CHIP-like [KO:K09561] [EC:2.3.2.27]
101698794  Ube2d1; ubiquitin conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
101724153  Ube2d2; ubiquitin conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
101725641  Ube2d3; ubiquitin conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
101725736  Ube2d4; ubiquitin conjugating enzyme E2 D4 (putative) [KO:K06689] [EC:2.3.2.23]
101700770  Park2; parkin RBR E3 ubiquitin protein ligase [KO:K04556] [EC:2.3.2.31]
101712475  Rbx1; ring-box 1 [KO:K03868] [EC:2.3.2.32]
101703786  Cul1; cullin 1 [KO:K03347]
101704606  Skp1; S-phase kinase associated protein 1 [KO:K03094]
101721920  Fbxo2; F-box protein 2 [KO:K10099]
101723682  Fbxo6; F-box 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
hgl00510  N-Glycan biosynthesis
hgl03050  Proteasome
hgl03060  Protein export
hgl04210  Apoptosis
hgl04930  Type II diabetes mellitus
hgl05010  Alzheimer disease
hgl05012  Parkinson disease
hgl05020  Prion disease
KO pathway
ko04141   
LinkDB

DBGET integrated database retrieval system