KEGG   PATHWAY: ngi04141
Entry
ngi04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Nannospalax galili (Upper Galilee mountains blind 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
ngi04141  Protein processing in endoplasmic reticulum
ngi04141

Other DBs
GO: 0030433 0034976
Organism
Nannospalax galili (Upper Galilee mountains blind mole rat) [GN:ngi]
Gene
103724406  Rnf5; ring finger protein 5 [KO:K10666] [EC:2.3.2.27]
103724413  Atf6b; activating transcription factor 6 beta [KO:K09049]
103724426  Hspa1l; heat shock protein family A (Hsp70) member 1 like [KO:K03283]
103724601  Atxn3; ataxin 3 [KO:K11863] [EC:3.4.22.-]
103724902  Map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
103724966  [KO:K09487]
103724975  Syvn1; synoviolin 1 [KO:K10601] [EC:2.3.2.27]
103724977  Capn1; calpain 1 [KO:K01367] [EC:3.4.22.52]
103725065  Man1c1; mannosidase alpha class 1C member 1 [KO:K01230] [EC:3.2.1.113]
103725718  Erlec1; endoplasmic reticulum lectin 1 [KO:K14008]
103725898  Ubqln1; ubiquilin 1 [KO:K04523]
103725931  Yod1; YOD1 deubiquitinase [KO:K13719] [EC:3.1.2.-]
103726159  Uggt2; UDP-glucose glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
103726160  Dnajc3; DnaJ heat shock protein family (Hsp40) member C3 [KO:K09523]
103726183  Dnajb11; DnaJ heat shock protein family (Hsp40) member B11 [KO:K09517]
103726218  Hspa4l; heat shock protein family A (Hsp70) member 4 like [KO:K09485]
103726266  Capn2; calpain 2 [KO:K03853] [EC:3.4.22.53]
103726522  Map2k7; mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
103726664  Edem3; ER degradation enhancing alpha-mannosidase like protein 3 [KO:K10086]
103726994  Mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
103727040  Hspa5; heat shock protein family A (Hsp70) member 5 [KO:K09490] [EC:3.6.4.10]
103727217  Hsph1; heat shock protein family H (Hsp110) member 1 [KO:K09485]
103727631  Hsp90aa1; heat shock protein 90 alpha family class A member 1 [KO:K04079]
103727752  Dnajb2; DnaJ heat shock protein family (Hsp40) member B2 [KO:K09508]
103727885  Hyou1; hypoxia up-regulated 1 [KO:K09486]
103727922  Hspa8; heat shock protein family A (Hsp70) member 8 [KO:K03283]
103728471  Edem2; ER degradation enhancing alpha-mannosidase like protein 2 [KO:K10085]
103728838  [KO:K06689] [EC:2.3.2.23]
103729122  Tmem258; transmembrane protein 258 [KO:K26502]
103729225  [KO:K04556] [EC:2.3.2.31]
103729340  Cryaa; crystallin alpha A [KO:K09541]
103729721  Sar1a; secretion associated Ras related GTPase 1A [KO:K07953] [EC:3.6.5.-]
103729775  Skp1; S-phase kinase associated protein 1 [KO:K03094]
103729839  Mbtps1; membrane bound transcription factor peptidase, site 1 [KO:K08653] [EC:3.4.21.112]
103729861  Sar1b; secretion associated Ras related GTPase 1B [KO:K07953] [EC:3.6.5.-]
103729867  Dnajc10; DnaJ heat shock protein family (Hsp40) member C10 [KO:K09530] [EC:1.8.4.-]
103729871  Sec24a; SEC24 homolog A, COPII coat complex component [KO:K14007]
103730145  Mbtps2; membrane bound transcription factor peptidase, site 2 [KO:K07765] [EC:3.4.24.85]
103730256  P4hb; prolyl 4-hydroxylase subunit beta [KO:K09580] [EC:5.3.4.1]
103730262  [KO:K08057]
103730341  Rpn1; ribophorin I [KO:K12666]
103730347  Sec61a1; Sec61 translocon alpha 1 subunit [KO:K10956]
103730366  Nploc4; NPL4 homolog, ubiquitin recognition factor [KO:K14015]
103730448  Sel1l; SEL1L ERAD E3 ligase adaptor subunit [KO:K14026]
103730676  Plaa; phospholipase A2 activating protein [KO:K14018]
103730742  Ube2d1; ubiquitin conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
103730766  Hsp90ab1; heat shock protein 90 alpha family class B member 1 [KO:K04079]
103730939  Ubqln2; ubiquilin 2 [KO:K04523]
103731195  Bcap31; B-cell receptor-associated protein 31 [KO:K14009]
103731199  Ssr4; signal sequence receptor subunit 4 [KO:K04571]
103731274  Dnaja2; DnaJ heat shock protein family (Hsp40) member A2 [KO:K09503]
103731381  Cul1; cullin 1 [KO:K03347]
103731393  Uggt1; UDP-glucose glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
103731707  Sec62; SEC62 homolog, preprotein translocation factor [KO:K12275]
103731954  Stt3b; STT3B, catalytic subunit of the oligosaccharyltransferase complex [KO:K07151] [EC:2.4.99.18]
103732072  [KO:K04523]
103732302  Sel1l2; SEL1L2 ERAD E3 ligase adaptor subunit [KO:K14026]
103732361  Lman2; lectin, mannose binding 2 [KO:K10082]
103732606  [KO:K23741]
103732819  Fbxo6; F-box only protein 6 [KO:K10100]
103732863  Fbxo2; F-box protein 2 [KO:K10099]
103732904  Txndc5; thioredoxin domain containing 5 [KO:K13984] [EC:5.3.4.1]
103732912  Ssr1; signal sequence receptor subunit 1 [KO:K13249]
103732983  Dnajc1; DnaJ heat shock protein family (Hsp40) member C1 [KO:K09521]
103733070  Ube4b; ubiquitination factor E4B [KO:K10597] [EC:2.3.2.27]
103733080  Ube2j1; ubiquitin conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
103733886  Cryab; crystallin alpha B [KO:K09542]
103734005  Sec23a; Sec23 homolog A, coat complex II component [KO:K14006]
103734033  Man1a2; mannosidase alpha class 1A member 2 [KO:K01230] [EC:3.2.1.113]
103734260  Ngly1; N-glycanase 1 [KO:K01456] [EC:3.5.1.52]
103734580  Prkcsh; protein kinase C substrate 80K-H [KO:K08288]
103734630  Ubxn4; UBX domain protein 4 [KO:K24348]
103734730  Pdia4; protein disulfide isomerase family A member 4 [KO:K09582] [EC:5.3.4.1]
103734968  Xbp1; X-box binding protein 1 [KO:K09027]
103734971  Ube2d4; ubiquitin conjugating enzyme E2 D4 (putative) [KO:K06689] [EC:2.3.2.23]
103735023  Mapk8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
103735086  Nsfl1c; NSFL1 cofactor [KO:K14012]
103735150  Sil1; SIL1 nucleotide exchange factor [KO:K14001]
103735593  Stt3a; STT3A, catalytic subunit of the oligosaccharyltransferase complex [KO:K07151] [EC:2.4.99.18]
103735595  Herpud1; homocysteine inducible ER protein with ubiquitin like domain 1 [KO:K14027]
103735677  Preb; prolactin regulatory element binding [KO:K14003]
103735703  Dnajc5g; DnaJ heat shock protein family (Hsp40) member C5 gamma [KO:K09525]
103736289  Man1a1; mannosidase alpha class 1A member 1 [KO:K01230] [EC:3.2.1.113]
103736922  Lman1l; lectin, mannose binding 1 like [KO:K10081]
103736940  Sec24c; SEC24 homolog C, COPII coat complex component [KO:K14007]
103736960  Dnajb12; DnaJ heat shock protein family (Hsp40) member B12 [KO:K09518]
103737054  Park2; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
103737226  Ubxn6; UBX domain protein 6 [KO:K14011]
103737614  Ssr3; signal sequence receptor subunit 3 [KO:K13251]
103737674  Ubxn1; UBX domain protein 1 [KO:K24348]
103737680  Ganab; glucosidase II alpha subunit [KO:K05546] [EC:3.2.1.207]
103737797  March6; membrane associated ring-CH-type finger 6 [KO:K10661] [EC:2.3.2.27]
103737830  Magt1; magnesium transporter 1 [KO:K19478]
103738157  Derl1; derlin 1 [KO:K11519]
103738565  Ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase non-catalytic subunit [KO:K12670]
103738699  Sec61a2; Sec61 translocon alpha 2 subunit [KO:K10956]
103739238  Ero1a; endoplasmic reticulum oxidoreductase 1 alpha [KO:K10950] [EC:1.8.4.-]
103739485  Dad1; defender against cell death 1 [KO:K12668]
103739617  Lman1; lectin, mannose binding 1 [KO:K10080]
103739733  Bak1; BCL2 antagonist/killer 1 [KO:K14021]
103739761  Sec23b; Sec23 homolog B, coat complex II component [KO:K14006]
103739946  Ube2g2; ubiquitin conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
103740069  Derl3; derlin 3 [KO:K13989]
103740099  Bcl2; BCL2, apoptosis regulator [KO:K02161]
103740131  Eif2ak3; eukaryotic translation initiation factor 2 alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
103740429  Rnf185; ring finger protein 185 [KO:K10666] [EC:2.3.2.27]
103740779  [KO:K04079]
103741172  Man1b1; mannosidase alpha class 1B member 1 [KO:K23741]
103741188  Traf2; TNF receptor associated factor 2 [KO:K03173] [EC:2.3.2.27]
103741554  Vcp; valosin containing protein [KO:K13525]
103741592  Bag1; BCL2 associated athanogene 1 [KO:K09555]
103741596  Dnaja1; DnaJ heat shock protein family (Hsp40) member A1 [KO:K09502]
103742066  Ufd1l; ubiquitin fusion degradation 1 like (yeast) [KO:K14016]
103742145  Hsp90b1; heat shock protein 90 beta family member 1 [KO:K09487]
103742166  Ckap4; cytoskeleton associated protein 4 [KO:K13999]
103742273  Dnajc5; DnaJ heat shock protein family (Hsp40) member C5 [KO:K09525]
103742502  Ostc; oligosaccharyltransferase complex non-catalytic subunit [KO:K26501]
103742679  Sec24b; SEC24 homolog B, COPII coat complex component [KO:K14007]
103742901  Os9; OS9, endoplasmic reticulum lectin [KO:K10088]
103742913  Ddit3; DNA damage inducible transcript 3 [KO:K04452]
103743082  Ube2d2; ubiquitin conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
103743283  Tusc3; tumor suppressor candidate 3 [KO:K19478]
103743488  Rbx1; ring-box 1 [KO:K03868] [EC:2.3.2.32]
103743586  Ubxn8; UBX domain protein 8 [KO:K24351]
103743605  Tram1l1; translocation associated membrane protein 1-like 1 [KO:K14010]
103743635  Ppp1r15a; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
103743639  Bax; BCL2 associated X, apoptosis regulator [KO:K02159]
103743862  [KO:K11519]
103743983  Sec31b; SEC31 homolog B, COPII coat complex component [KO:K14005]
103744301  Sec24d; SEC24 homolog D, COPII coat complex component [KO:K14007]
103744671  Bag2; BCL2 associated athanogene 2 [KO:K09556]
103744745  Ern1; endoplasmic reticulum to nucleus signaling 1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
103744818  Hspbp1; HSPA (Hsp70) binding protein 1 [KO:K09562]
103744990  Sec31a; SEC31 homolog A, COPII coat complex component [KO:K14005]
103745567  Erp29; endoplasmic reticulum protein 29 [KO:K09586]
103745838  [KO:K06689] [EC:2.3.2.23]
103745996  [KO:K03283]
103746582  Ubxn2a; UBX domain protein 2A [KO:K24349]
103746605  Tram1; translocation associated membrane protein 1 [KO:K14010]
103746706  Pdia3; protein disulfide isomerase family A member 3 [KO:K08056] [EC:5.3.4.1]
103746770  Hspa6; heat shock protein family A (Hsp70) member 6 [KO:K03283]
103746777  Atf6; activating transcription factor 6 [KO:K09054]
103747195  Selenos; selenoprotein S [KO:K14025]
103747612  Pdia6; protein disulfide isomerase family A member 6 [KO:K09584] [EC:5.3.4.1]
103747722  Dnajc5b; DnaJ heat shock protein family (Hsp40) member C5 beta [KO:K09525]
103747734  Rrbp1; ribosome binding protein 1 [KO:K14000]
103747770  Sec63; SEC63 homolog, protein translocation regulator [KO:K09540]
103748155  Svip; small VCP interacting protein [KO:K14014]
103748563  Atf4; activating transcription factor 4 [KO:K04374]
103748851  Sec61g; Sec61 translocon gamma subunit [KO:K07342]
103748972  Mapk10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
103749081  Sec61b; Sec61 translocon beta subunit [KO:K09481]
103749287  Eif2s1; eukaryotic translation initiation factor 2 subunit alpha [KO:K03237]
103749366  Mapk9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
103749377  Canx; calnexin [KO:K08054]
103749739  Ube2j2; ubiquitin conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
103749857  Amfr; autocrine motility factor receptor [KO:K10636] [EC:2.3.2.36]
103750637  Wfs1; wolframin ER transmembrane glycoprotein [KO:K14020]
103750657  Ubqln4; ubiquilin 4 [KO:K04523]
103750666  Ssr2; signal sequence receptor subunit 2 [KO:K13250]
103751010  Krtcap2; keratinocyte associated protein 2 [KO:K28202]
103751206  Edem1; ER degradation enhancing alpha-mannosidase like protein 1 [KO:K10084]
103751260  Rad23b; RAD23 homolog B, nucleotide excision repair protein [KO:K10839]
103751437  Sec13; SEC13 homolog, nuclear pore and COPII coat complex component [KO:K14004]
103751574  Ero1b; endoplasmic reticulum oxidoreductase 1 beta [KO:K10976] [EC:1.8.4.-]
103751782  Nfe2l2; nuclear factor, erythroid 2 like 2 [KO:K05638]
103751843  Dnajb1; DnaJ heat shock protein family (Hsp40) member B1 [KO:K09507]
103751873  Rad23a; RAD23 homolog A, nucleotide excision repair protein [KO:K10839]
103751874  Calr; calreticulin [KO:K08057]
103751994  Ube2g1; ubiquitin conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
103752032  Rpn2; ribophorin II [KO:K12667]
103752215  Derl2; derlin 2 [KO:K13989]
103752392  Stub1; STIP1 homology and U-box containing protein 1 [KO:K09561] [EC:2.3.2.27]
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
ngi00510  N-Glycan biosynthesis
ngi03050  Proteasome
ngi03060  Protein export
ngi04210  Apoptosis
ngi04930  Type II diabetes mellitus
ngi05010  Alzheimer disease
ngi05012  Parkinson disease
ngi05020  Prion disease
KO pathway
ko04141   

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