KEGG   PATHWAY: setr04141
Entry
setr04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Suncus etruscus (white-toothed pygmy shrew)
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
setr04141  Protein processing in endoplasmic reticulum
setr04141

Other DBs
GO: 0030433 0034976
Organism
Suncus etruscus (white-toothed pygmy shrew) [GN:setr]
Gene
125994856  SEC31B; LOW QUALITY PROTEIN: protein transport protein Sec31B [KO:K14005]
125995123  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
125995703  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
125996027  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
125996178  SSR1; translocon-associated protein subunit alpha [KO:K13249]
125996185  [KO:K03283]
125996186  [KO:K03283]
125996188  [KO:K03283]
125996226  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
125996306  [KO:K04079]
125996343  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
125996555  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
125996569  PRKN; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
125997349  DERL1; derlin-1 [KO:K11519]
125997473  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
125997509  [KO:K13989]
125997875  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
125997930  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
125998074  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
125998123  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
125998239  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
125998247  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
125998462  SEC23A; protein transport protein Sec23A [KO:K14006]
125998726  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
125999188  UBQLN2; ubiquilin-2 [KO:K04523]
125999249  MAGT1; magnesium transporter protein 1 isoform X1 [KO:K19478]
125999463  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
125999474  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
125999693  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
126000772  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
126000835  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
126001339  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
126001450  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
126001621  UGGT1; LOW QUALITY PROTEIN: UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
126001960  [KO:K03094]
126001977  [KO:K03094]
126002926  CUL1; cullin-1 [KO:K03347]
126003282  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
126003536  LMAN1L; protein ERGIC-53-like [KO:K10081]
126003543  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
126003592  ATXN3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
126003593  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
126003698  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
126003830  [KO:K07342]
126003897  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
126003989  SEL1L; protein sel-1 homolog 1 [KO:K14026]
126004063  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
126004186  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
126004421  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
126004511  [KO:K03094]
126005116  DERL2; derlin-2 [KO:K13989]
126005122  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
126005473  PLAA; phospholipase A-2-activating protein [KO:K14018]
126005864  SEC63; translocation protein SEC63 homolog [KO:K09540]
126006026  MAN1A1; LOW QUALITY PROTEIN: mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
126006324  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
126006345  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
126006569  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
126006744  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
126006847  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
126006863  UBXN8; UBX domain-containing protein 8 [KO:K24351]
126006888  [KO:K07342]
126008924  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
126009063  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
126009181  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
126009226  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
126009253  UBXN4; UBX domain-containing protein 4 [KO:K24348]
126009254  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
126009406  HSPA5; LOW QUALITY PROTEIN: endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
126009645  [KO:K06689] [EC:2.3.2.23]
126010738  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
126010760  SEL1L2; protein sel-1 homolog 2 isoform X1 [KO:K14026]
126010791  SEC23B; protein transport protein Sec23B [KO:K14006]
126010853  SEC62; translocation protein SEC62 [KO:K12275]
126010922  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
126011067  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
126011470  SSR3; translocon-associated protein subunit gamma [KO:K13251]
126011500  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
126011562  FBXO2; F-box only protein 2 [KO:K10099]
126011570  [KO:K10100]
126011571  [KO:K10100]
126011596  [KO:K10100]
126011618  [KO:K10100]
126011963  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
126011964  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
126013369  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
126013393  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
126013535  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
126013575  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
126013597  [KO:K07342]
126013732  [KO:K03283]
126013762  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
126013878  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
126013950  NPLOC4; LOW QUALITY PROTEIN: nuclear protein localization protein 4 homolog [KO:K14015]
126013969  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
126014164  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
126014517  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
126015401  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
126015589  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
126015645  CANX; calnexin isoform X1 [KO:K08054]
126015663  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
126016002  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
126016036  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
126016120  CRYAB; alpha-crystallin B chain [KO:K09542]
126016156  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
126016203  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
126016286  [KO:K03094]
126016454  [KO:K04741] [EC:3.4.22.-]
126016565  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
126017312  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
126017325  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
126017387  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
126017457  [KO:K11863] [EC:3.4.22.-]
126017599  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
126017821  UBXN1; UBX domain-containing protein 1 [KO:K24348]
126017878  TMEM258; transmembrane protein 258 [KO:K26502]
126017986  [KO:K03283]
126018030  SYVN1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
126018055  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
126018146  [KO:K04523]
126018993  SVIP; small VCP/p97-interacting protein [KO:K14014]
126020367  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
126020385  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
126020554  [KO:K03094]
126020568  LMAN1; protein ERGIC-53 [KO:K10080]
126020600  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
126020708  UBQLN4; ubiquilin-4 [KO:K04523]
126020766  SSR2; translocon-associated protein subunit beta [KO:K13250]
126020770  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
126020890  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
126020935  WFS1; wolframin [KO:K14020]
126020945  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
126021328  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
126021847  SELENOS; selenoprotein S [KO:K14025]
126022223  OS9; protein OS-9 [KO:K10088]
126022241  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
126022257  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
126022286  HSP90B1; endoplasmin [KO:K09487]
126023521  [KO:K07765] [EC:3.4.24.85]
126023825  PREB; prolactin regulatory element-binding protein [KO:K14003]
126023924  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
126024044  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
126024075  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
126024288  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
126024396  ERLEC1; LOW QUALITY PROTEIN: endoplasmic reticulum lectin 1 [KO:K14008]
126024479  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
126025658  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
126026111  [KO:K03094]
126027036  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
126027057  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
126027091  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
126027154  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
126027192  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
126027215  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
126027261  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
126027547  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
126027711  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
126027713  MBTPS1; LOW QUALITY PROTEIN: membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
126027774  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
126027783  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
126027833  SEC24D; protein transport protein Sec24D [KO:K14007]
126027835  SEC24B; protein transport protein Sec24B [KO:K14007]
126027895  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
126027929  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
126028014  HSPBP1; hsp70-binding protein 1 [KO:K09562]
126029694  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
126030251  CALR; calreticulin [KO:K08057]
126030253  RAD23A; UV excision repair protein RAD23 homolog A [KO:K10839]
126030331  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
126030351  PRKCSH; glucosidase 2 subunit beta [KO:K08288]
126030755  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
126030775  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
126030804  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
126030844  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
126030907  DNAJB12; dnaJ homolog subfamily B member 12 [KO:K09518]
126030949  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
126031093  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
126031120  UBXN6; UBX domain-containing protein 6 [KO:K14011]
126031156  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
126032054  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
126032439  [KO:K03347]
126032587  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
126032651  [KO:K03868] [EC:2.3.2.32]
126033554  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
126033625  [KO:K09502]
126033690  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
126033732  DERL3; derlin-3 [KO:K13989]
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
setr00510  N-Glycan biosynthesis
setr03050  Proteasome
setr03060  Protein export
setr04210  Apoptosis
setr04930  Type II diabetes mellitus
setr05010  Alzheimer disease
setr05012  Parkinson disease
setr05020  Prion disease
KO pathway
ko04141   

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