KEGG   PATHWAY: phas04141
Entry
phas04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Phyllostomus hastatus (greater spear-nosed bat)
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
phas04141  Protein processing in endoplasmic reticulum
phas04141

Other DBs
GO: 0030433 0034976
Organism
Phyllostomus hastatus (greater spear-nosed bat) [GN:phas]
Gene
123804424  SSR2; translocon-associated protein subunit beta isoform X1 [KO:K13250]
123804434  UBQLN4; ubiquilin-4 [KO:K04523]
123804516  KRTCAP2; keratinocyte-associated protein 2 isoform X1 [KO:K28202]
123804531  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
123805836  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
123805888  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
123805930  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
123806044  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
123806609  DERL3; derlin-3 isoform X1 [KO:K13989]
123806667  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
123806762  [KO:K14005]
123806844  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
123806861  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
123806902  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
123806937  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
123807193  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
123807455  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
123807480  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
123807491  TUSC3; tumor suppressor candidate 3 [KO:K19478]
123807598  UBXN8; UBX domain-containing protein 8 [KO:K24351]
123808077  SEL1L; protein sel-1 homolog 1 [KO:K14026]
123808178  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
123808305  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
123808406  ATXN3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
123808632  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
123808737  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
123808900  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
123809002  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
123809201  DERL2; derlin-2 isoform X1 [KO:K13989]
123809349  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
123809712  SELENOS; selenoprotein S [KO:K14025]
123810059  [KO:K14014]
123810236  CANX; calnexin [KO:K08054]
123810273  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
123810362  [KO:K03283]
123810388  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
123810420  UBXN4; UBX domain-containing protein 4 [KO:K24348]
123810486  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
123810504  [KO:K03283]
123810694  HSP90B1; endoplasmin [KO:K09487]
123810736  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
123810932  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
123811000  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
123811410  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
123811418  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
123811663  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
123811997  UBE2D4; ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
123812054  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
123812775  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
123812892  OS9; protein OS-9 isoform X1 [KO:K10088]
123813217  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
123813362  HSPBP1; hsp70-binding protein 1 [KO:K09562]
123813410  [KO:K09481]
123813690  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
123813794  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
123813823  SEC24D; protein transport protein Sec24D [KO:K14007]
123814594  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
123814691  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
123814791  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
123814802  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
123814894  SEC23B; protein transport protein Sec23B [KO:K14006]
123815022  UBQLN2; ubiquilin-2 [KO:K04523]
123815101  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
123815132  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
123815351  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
123815585  CUL1; cullin-1 [KO:K03347]
123815629  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
123815892  WFS1; wolframin [KO:K14020]
123816126  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
123816194  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
123816219  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
123816301  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
123816900  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
123816901  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
123816997  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
123818346  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
123818349  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
123818526  [KO:K04741] [EC:3.4.22.-]
123818558  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
123818737  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
123818781  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
123819310  [KO:K04523]
123819619  CRYAB; alpha-crystallin B chain [KO:K09542]
123819957  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
123820007  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
123820015  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
123820050  [KO:K13525]
123820109  PLAA; phospholipase A-2-activating protein [KO:K14018]
123820151  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
123820190  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
123820468  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
123820563  YOD1; ubiquitin thioesterase OTU1 isoform X1 [KO:K13719] [EC:3.1.2.-]
123820597  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
123820721  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
123820722  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
123820733  [KO:K07342]
123820887  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
123820920  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
123820985  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
123820994  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
123821069  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
123821362  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
123821433  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
123821582  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
123821596  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
123821674  SEC63; translocation protein SEC63 homolog [KO:K09540]
123821698  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
123822135  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
123822404  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
123822439  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
123822465  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
123822599  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
123822646  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
123823097  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
123823110  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
123823431  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
123823586  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
123823598  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
123823698  [KO:K26502]
123824279  [KO:K06689] [EC:2.3.2.23]
123824280  [KO:K06689] [EC:2.3.2.23]
123824332  [KO:K06689] [EC:2.3.2.23]
123824344  [KO:K06689] [EC:2.3.2.23]
123824345  [KO:K06689] [EC:2.3.2.23]
123824348  [KO:K06689] [EC:2.3.2.23]
123824349  [KO:K06689] [EC:2.3.2.23]
123824350  [KO:K06689] [EC:2.3.2.23]
123824351  [KO:K06689] [EC:2.3.2.23]
123824369  [KO:K06689] [EC:2.3.2.23]
123824376  [KO:K06689] [EC:2.3.2.23]
123824377  [KO:K06689] [EC:2.3.2.23]
123824379  MAGT1; magnesium transporter protein 1 isoform X1 [KO:K19478]
123824593  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 [KO:K10661] [EC:2.3.2.27]
123824878  [KO:K07342]
123825159  LMAN1L; LOW QUALITY PROTEIN: protein ERGIC-53-like [KO:K10081]
123825261  SEC23A; protein transport protein Sec23A [KO:K14006]
123825511  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
123826047  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
123826111  LMAN1; protein ERGIC-53 [KO:K10080]
123826251  SEC61A1; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
123826397  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
123826398  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
123826609  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
123826987  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
123827215  SEC62; translocation protein SEC62 [KO:K12275]
123827223  SSR3; translocon-associated protein subunit gamma [KO:K13251]
123828000  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
123828020  CRYAA; alpha-crystallin A chain isoform X1 [KO:K09541]
123828193  CALR; calreticulin isoform X1 [KO:K08057]
123828265  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
123828282  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
123828410  RAD23A; UV excision repair protein RAD23 homolog A [KO:K10839]
123828949  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
123829131  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
123829347  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
123829348  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
123829399  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
123829406  UBXN1; UBX domain-containing protein 1 [KO:K24348]
123829491  TMEM258; transmembrane protein 258 [KO:K26502]
123830011  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
123830012  YY2; transcription factor YY2 [KO:K07765] [EC:3.4.24.85]
123830279  BAX; apoptosis regulator BAX [KO:K02159]
123830343  PPP1R15A; protein phosphatase 1 regulatory subunit 15A isoform X1 [KO:K14019]
123830388  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
123830472  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
123830576  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
123831121  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
123831324  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
123831620  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
123831671  [KO:K10100]
123831740  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
123831744  [KO:K10100]
123832015  FBXO2; F-box only protein 2 [KO:K10099]
123832060  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
123832119  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
123832543  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
123832579  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
123832759  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
123833037  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
123833151  DERL1; derlin-1 [KO:K11519]
123833204  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
123833256  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
123833466  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
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
phas00510  N-Glycan biosynthesis
phas03050  Proteasome
phas03060  Protein export
phas04210  Apoptosis
phas04930  Type II diabetes mellitus
phas05010  Alzheimer disease
phas05012  Parkinson disease
phas05020  Prion disease
KO pathway
ko04141   

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