KEGG   PATHWAY: ppam04141
Entry
ppam04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Pteronotus mesoamericanus (Parnell's mustached 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
ppam04141  Protein processing in endoplasmic reticulum
ppam04141

Other DBs
GO: 0030433 0034976
Organism
Pteronotus mesoamericanus (Parnell's mustached bat) [GN:ppam]
Gene
129061243  SELENOS; selenoprotein S isoform X1 [KO:K14025]
129061394  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
129061433  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
129061480  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
129061765  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
129061823  UBXN4; UBX domain-containing protein 4 [KO:K24348]
129061892  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
129062028  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
129062375  SSR2; translocon-associated protein subunit beta [KO:K13250]
129062386  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
129062447  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
129062451  UBQLN4; ubiquilin-4 [KO:K04523]
129062532  CALR; calreticulin [KO:K08057]
129062585  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
129062613  SEC24D; protein transport protein Sec24D [KO:K14007]
129062755  TMEM258; transmembrane protein 258 [KO:K26502]
129062959  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
129063290  CUL1; cullin-1 [KO:K03347]
129063758  [KO:K03283]
129063835  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
129064295  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
129064461  [KO:K09502]
129064612  UBQLN2; ubiquilin-2 [KO:K04523]
129064693  TUSC3; tumor suppressor candidate 3 [KO:K19478]
129064837  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
129064912  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
129064999  CANX; calnexin [KO:K08054]
129065007  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
129065155  [KO:K04079]
129065320  [KO:K06689] [EC:2.3.2.23]
129065325  [KO:K06689] [EC:2.3.2.23]
129065379  [KO:K06689] [EC:2.3.2.23]
129065380  [KO:K06689] [EC:2.3.2.23]
129065381  [KO:K06689] [EC:2.3.2.23]
129065382  [KO:K06689] [EC:2.3.2.23]
129065383  [KO:K06689] [EC:2.3.2.23]
129065384  [KO:K06689] [EC:2.3.2.23]
129065402  MAGT1; magnesium transporter protein 1 [KO:K19478]
129065823  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
129065851  SEL1L; protein sel-1 homolog 1 [KO:K14026]
129065952  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
129066033  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
129066210  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
129066636  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
129066645  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
129066678  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
129066679  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
129066684  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
129066720  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
129066979  [KO:K03283]
129066993  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
129066998  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
129067324  [KO:K03283]
129067339  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
129067340  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
129067345  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
129067491  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
129067549  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
129067611  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
129067679  SEC63; translocation protein SEC63 homolog [KO:K09540]
129067738  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
129067800  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
129067941  [KO:K26501]
129068192  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
129068223  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
129068228  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
129068584  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
129068585  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
129068640  UBXN1; UBX domain-containing protein 1 [KO:K24348]
129068645  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
129068848  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
129068939  UBE2D4; ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
129069141  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
129069190  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
129069687  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
129069866  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
129069894  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
129069918  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
129069974  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
129070208  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
129070721  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
129070746  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
129070800  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
129070891  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
129070901  SEC23B; protein transport protein Sec23B [KO:K14006]
129070975  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
129071209  [KO:K06689] [EC:2.3.2.23]
129071358  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
129071373  HSP90B1; endoplasmin [KO:K09487]
129071762  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
129072057  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
129072103  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
129072359  DERL2; derlin-2 isoform X1 [KO:K13989]
129072373  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
129072767  CRYAA; alpha-crystallin A chain isoform X1 [KO:K09541]
129072993  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
129073479  HSPH1; heat shock protein 105 kDa [KO:K09485]
129073854  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
129074033  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
129074334  SSR4; translocon-associated protein subunit delta [KO:K04571]
129074355  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
129074435  [KO:K11863] [EC:3.4.22.-]
129074535  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 [KO:K10661] [EC:2.3.2.27]
129074572  [KO:K06689] [EC:2.3.2.23]
129075092  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
129075133  LMAN1L; LOW QUALITY PROTEIN: protein ERGIC-53-like [KO:K10081]
129075151  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
129075387  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
129075598  FBXO2; F-box only protein 2 [KO:K10099]
129075653  FBXO6; F-box only protein 6 [KO:K10100]
129075669  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
129075791  SSR3; translocon-associated protein subunit gamma [KO:K13251]
129075812  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
129075820  OS9; protein OS-9 isoform X1 [KO:K10088]
129076220  SEC62; translocation protein SEC62 [KO:K12275]
129076239  [KO:K04079]
129076501  [KO:K04523]
129076591  [KO:K03094]
129076927  [KO:K04079]
129076938  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
129077113  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
129077517  WFS1; wolframin [KO:K14020]
129077781  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
129077836  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
129077850  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
129078161  UBXN6; UBX domain-containing protein 6 [KO:K14011]
129078190  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
129078216  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
129078238  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
129078475  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
129078555  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
129078753  LMAN1; protein ERGIC-53 [KO:K10080]
129078764  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
129079150  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
129079269  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
129079274  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
129079369  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
129079397  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
129079528  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
129079857  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
129079949  [KO:K04079]
129080324  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
129080502  SVIP; small VCP/p97-interacting protein [KO:K14014]
129080730  [KO:K04079]
129080940  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
129080941  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
129081242  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
129081762  [KO:K26501]
129081805  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 isoform X1 [KO:K12666]
129081810  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
129081977  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
129082078  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
129082282  [KO:K04079]
129082353  [KO:K03283]
129082425  PLAA; phospholipase A-2-activating protein [KO:K14018]
129082608  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
129082913  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
129082924  BAX; apoptosis regulator BAX [KO:K02159]
129083074  [KO:K03283]
129083685  SEC23A; protein transport protein Sec23A [KO:K14006]
129083921  [KO:K04079]
129084012  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
129084197  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
129084304  DERL1; derlin-1 [KO:K11519]
129085039  [KO:K10839]
129085292  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
129085297  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
129085319  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
129085376  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
129085414  [KO:K03283]
129085523  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
129085527  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
129085559  EIF2AK3; LOW QUALITY PROTEIN: eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
129085732  [KO:K03283]
129085846  [KO:K04079]
129085920  [KO:K04079]
129085949  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
129086049  CRYAB; alpha-crystallin B chain [KO:K09542]
129086067  [KO:K04741] [EC:3.4.22.-]
129086224  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
129086317  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
129086368  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
129086385  DERL3; derlin-3 [KO:K13989]
129086426  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
129086699  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
129086807  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
129086808  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
129087130  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
129087257  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
129087374  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
129087587  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
129088228  PRKN; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
129088272  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
129088396  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
129088399  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
129088420  [KO:K06689] [EC:2.3.2.23]
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
ppam00510  N-Glycan biosynthesis
ppam03050  Proteasome
ppam03060  Protein export
ppam04210  Apoptosis
ppam04930  Type II diabetes mellitus
ppam05010  Alzheimer disease
ppam05012  Parkinson disease
ppam05020  Prion disease
KO pathway
ko04141   

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