KEGG   PATHWAY: tpai04141
Entry
tpai04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Tympanuchus pallidicinctus (lesser prairie-chicken)
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
tpai04141  Protein processing in endoplasmic reticulum
tpai04141

Other DBs
GO: 0030433 0034976
Organism
Tympanuchus pallidicinctus (lesser prairie-chicken) [GN:tpai]
Gene
128071899  SSR2; translocon-associated protein subunit beta [KO:K13250]
128071986  MOGS; mannosyl-oligosaccharide glucosidase isoform X1 [KO:K01228] [EC:3.2.1.106]
128072026  UBQLN4; ubiquilin-4 [KO:K04523]
128072155  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
128072269  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
128072934  [KO:K08288]
128073549  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
128073589  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
128073610  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
128073663  HSP90B1; endoplasmin [KO:K09487]
128074100  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
128074322  LMAN1; protein ERGIC-53 [KO:K10080]
128074605  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
128074859  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
128075303  SELENOS; selenoprotein S isoform X1 [KO:K14025]
128075352  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
128075434  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
128076007  MAGT1; magnesium transporter protein 1 isoform X1 [KO:K19478]
128076189  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
128076307  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
128076505  CRYAA; alpha-crystallin A chain [KO:K09541]
128076633  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
128076751  UBXN4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
128076768  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
128077187  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
128077209  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
128077469  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
128077700  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
128077895  DNAJB12; dnaJ homolog subfamily B member 12 [KO:K09518]
128077939  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
128077983  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
128078150  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
128078448  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
128078478  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
128078517  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
128078708  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
128078817  [KO:K04452]
128078974  ERN2; serine/threonine-protein kinase/endoribonuclease IRE2 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
128079095  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
128079104  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
128079321  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA isoform X1 [KO:K01230] [EC:3.2.1.113]
128079446  [KO:K08288]
128079734  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
128079770  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741]
128079775  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
128079904  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
128079966  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
128079985  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
128079999  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
128080220  WFS1; wolframin isoform X1 [KO:K14020]
128080321  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
128080653  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
128080686  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
128080696  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
128080859  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
128080923  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
128080936  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
128080954  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
128081014  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
128081032  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
128081167  [KO:K06689] [EC:2.3.2.23]
128081169  [KO:K06689] [EC:2.3.2.23]
128081170  [KO:K06689] [EC:2.3.2.23]
128081171  [KO:K06689] [EC:2.3.2.23]
128081172  [KO:K06689] [EC:2.3.2.23]
128081377  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
128081465  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
128081555  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
128081639  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
128081678  CANX; calnexin isoform X1 [KO:K08054]
128081721  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
128081733  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
128081767  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
128081892  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
128081919  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
128082192  SSR3; translocon-associated protein subunit gamma [KO:K13251]
128082230  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
128082488  PREB; prolactin regulatory element-binding protein [KO:K14003]
128082734  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
128082743  SEC62; translocation protein SEC62 [KO:K12275]
128082893  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
128083046  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
128083281  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
128083462  DERL2; derlin-2 [KO:K13989]
128083479  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
128083494  UBXN2A; LOW QUALITY PROTEIN: UBX domain-containing protein 2A [KO:K24349]
128083618  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
128083876  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
128083879  SEC63; translocation protein SEC63 homolog [KO:K09540]
128083882  XBP1; X-box-binding protein 1 isoform X1 [KO:K09027]
128083906  [KO:K13989]
128083975  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
128084095  SEC24A; protein transport protein Sec24A [KO:K14007]
128084102  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
128084146  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
128084207  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
128084457  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
128084658  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
128084713  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
128084836  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
128084947  [KO:K10100]
128084948  FBXO2; F-box only protein 2 isoform X1 [KO:K10099]
128084964  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
128084979  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
128085168  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
128085291  HSP90AB1; heat shock protein HSP 90-beta isoform X1 [KO:K04079]
128085411  CAPN11; calpain-11 isoform X1 [KO:K01367] [EC:3.4.22.52]
128085715  SEL1L; protein sel-1 homolog 1 [KO:K14026]
128085989  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
128086120  SVIP; small VCP/p97-interacting protein [KO:K14014]
128086261  SEC23A; protein transport protein Sec23A [KO:K14006]
128086496  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
128086885  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
128087059  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
128087130  TMEM258; transmembrane protein 258 isoform X1 [KO:K26502]
128087210  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
128087351  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
128087513  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
128087575  CRYAB; alpha-crystallin B chain [KO:K09542]
128087617  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
128087642  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
128087755  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
128087775  CUL1; cullin-1 isoform X1 [KO:K03347]
128087778  PDIA4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
128087912  TXNDC5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
128087936  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
128088079  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
128088157  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
128088378  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
128088434  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
128088691  [KO:K04523]
128088882  CALR3; calreticulin-3 isoform X1 [KO:K08057]
128089353  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
128089385  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
128089438  PLAA; phospholipase A-2-activating protein [KO:K14018]
128089545  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
128089751  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
128089834  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
128090445  UBXN6; UBX domain-containing protein 6 [KO:K14011]
128090652  [KO:K09502]
128091007  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
128091068  DERL1; derlin-1 [KO:K11519]
128091078  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
128091169  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
128091446  BAG1; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555]
128091594  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
128091729  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
128092206  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
128092239  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
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
tpai00510  N-Glycan biosynthesis
tpai03050  Proteasome
tpai03060  Protein export
tpai04210  Apoptosis
KO pathway
ko04141   

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