KEGG   PATHWAY: pgig04141
Entry
pgig04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Pteropus vampyrus (Indian flying fox)
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
pgig04141  Protein processing in endoplasmic reticulum
pgig04141

Other DBs
GO: 0030433 0034976
Organism
Pteropus vampyrus (Indian flying fox) [GN:pgig]
Gene
120581290  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
120581300  SEC24A; LOW QUALITY PROTEIN: protein transport protein Sec24A [KO:K14007]
120581301  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
120581363  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
120581390  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
120581643  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
120582413  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
120582424  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
120582425  DNAJC3; dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
120582866  WFS1; wolframin isoform X1 [KO:K14020]
120583836  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
120584186  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
120584396  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
120584624  LMAN1; protein ERGIC-53 [KO:K10080]
120584867  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
120584877  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
120585094  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
120585450  DNAJC5B; dnaJ homolog subfamily C member 5B isoform X1 [KO:K09525]
120585475  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
120585817  FBXO6; F-box only protein 6 isoform X1 [KO:K10100]
120585819  FBXO2; F-box only protein 2 [KO:K10099]
120585840  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
120586271  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
120586800  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
120587360  SELENOS; selenoprotein S [KO:K14025]
120587686  DNAJB2; dnaJ homolog subfamily B member 2 [KO:K09508]
120588276  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
120588349  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
120589050  UBXN8; UBX domain-containing protein 8 [KO:K24351]
120589322  [KO:K06689] [EC:2.3.2.23]
120589352  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
120590116  UBQLN3; ubiquilin-3 [KO:K04523]
120591109  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
120591142  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
120591222  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
120591544  BCAP29; B-cell receptor-associated protein 29 [KO:K28771]
120591682  MAGT1; magnesium transporter protein 1 [KO:K19478]
120592140  KRTCAP2; LOW QUALITY PROTEIN: keratinocyte-associated protein 2 [KO:K28202]
120592217  SSR2; translocon-associated protein subunit beta [KO:K13250]
120592239  UBQLN4; ubiquilin-4 [KO:K04523]
120592487  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
120592620  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
120593001  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
120593538  UBQLN2; ubiquilin-2 [KO:K04523]
120593960  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
120594265  YY2; transcription factor YY2 [KO:K07765] [EC:3.4.24.85]
120594269  [KO:K07342]
120594554  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
120594611  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
120594822  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
120594953  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
120595544  DERL1; derlin-1 isoform X1 [KO:K11519]
120595966  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
120596182  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
120596273  [KO:K06689] [EC:2.3.2.23]
120596304  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
120597062  [KO:K14016]
120597087  NFE2L2; LOW QUALITY PROTEIN: nuclear factor erythroid 2-related factor 2 [KO:K05638]
120597149  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
120597199  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
120597665  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
120597781  LMAN1L; protein ERGIC-53-like isoform X1 [KO:K10081]
120597877  SEC23A; protein transport protein Sec23A [KO:K14006]
120598352  HSPBP1; hsp70-binding protein 1 [KO:K09562]
120598668  CRYAA; alpha-crystallin A chain isoform X1 [KO:K09541]
120598677  [KO:K13251]
120598678  [KO:K13251]
120598851  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
120599677  [KO:K04741] [EC:3.4.22.-]
120599784  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
120599824  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741]
120600114  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
120600206  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
120600274  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
120600654  TMEM258; transmembrane protein 258 [KO:K26502]
120600838  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
120600845  UBXN1; UBX domain-containing protein 1 [KO:K24348]
120601485  CUL1; cullin-1 [KO:K03347]
120601509  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
120601748  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:5.6.1.10]
120601994  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
120602007  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
120602095  HSPH1; heat shock protein 105 kDa [KO:K09485]
120602480  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
120602973  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
120602997  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
120603483  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
120603582  [KO:K10085]
120603631  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
120603965  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
120604370  SEC62; translocation protein SEC62 [KO:K12275]
120604424  ERO1A; ERO1-like protein alpha isoform X1 [KO:K10950] [EC:1.8.4.-]
120604962  DDIT3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452]
120604966  OS9; protein OS-9 isoform X1 [KO:K10088]
120605008  [KO:K14014]
120605692  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
120605906  [KO:K04571]
120605929  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
120605930  [KO:K04571]
120606720  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
120606862  DERL3; derlin-3 isoform X1 [KO:K13989]
120606950  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
120606962  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
120607669  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
120607674  SSR1; translocon-associated protein subunit alpha [KO:K13249]
120607897  DERL2; derlin-2 isoform X1 [KO:K13989]
120608084  SEC63; translocation protein SEC63 homolog [KO:K09540]
120608152  [KO:K06689] [EC:2.3.2.23]
120608719  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
120609165  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
120609197  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
120609382  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
120609485  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
120609518  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
120609536  SEC24C; LOW QUALITY PROTEIN: protein transport protein Sec24C [KO:K14007]
120609636  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
120610458  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
120610520  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
120610831  PRKCSH; glucosidase 2 subunit beta [KO:K08288]
120611863  SEC23B; protein transport protein Sec23B [KO:K14006]
120611877  RRBP1; LOW QUALITY PROTEIN: ribosome-binding protein 1 [KO:K14000]
120611891  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
120611942  UBXN4; UBX domain-containing protein 4 [KO:K24348]
120612131  SEC61A1; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
120612139  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
120612399  HSPA8; heat shock cognate 71 kDa protein isoform X1 [KO:K03283]
120612759  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
120612876  [KO:K13525]
120612919  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
120612956  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
120612967  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
120613413  [KO:K26502]
120614089  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
120614598  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
120614666  DNAJC1; LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 1 [KO:K09521]
120614917  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
120614942  HSP90B1; endoplasmin [KO:K09487]
120615393  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
120615779  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
120616195  SEC24D; protein transport protein Sec24D [KO:K14007]
120616245  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
120616340  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
120616563  LMAN2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
120616614  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
120616625  CANX; calnexin [KO:K08054]
120617101  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
120617232  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
120617300  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
120617310  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
120617538  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
120617559  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
120618006  [KO:K26502]
120618468  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
120618527  CALR; calreticulin [KO:K08057]
120618528  RAD23A; UV excision repair protein RAD23 homolog A [KO:K10839]
120618884  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
120618891  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
120618906  HSPA1B; heat shock 70 kDa protein 1B [KO:K03283]
120618910  HSPA1A; heat shock 70 kDa protein 1A [KO:K03283]
120618911  [KO:K03283]
120619310  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
120619577  SEL1L; protein sel-1 homolog 1 [KO:K14026]
120619620  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
120619712  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
120620196  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
120621094  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
120621541  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
120621651  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
120621853  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
120621858  OST4; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 4 [KO:K00730]
120621903  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
120621960  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
120621991  CRYAB; alpha-crystallin B chain isoform X1 [KO:K09542]
120622712  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
120622951  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
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
pgig00510  N-Glycan biosynthesis
pgig03050  Proteasome
pgig03060  Protein export
pgig04210  Apoptosis
pgig04930  Type II diabetes mellitus
pgig05010  Alzheimer disease
pgig05012  Parkinson disease
pgig05020  Prion disease
KO pathway
ko04141   

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