KEGG   PATHWAY: bbuf04141
Entry
bbuf04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Bufo bufo (common toad)
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
bbuf04141  Protein processing in endoplasmic reticulum
bbuf04141

Other DBs
GO: 0030433 0034976
Organism
Bufo bufo (common toad) [GN:bbuf]
Gene
120979686  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
121001510  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
121002030  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
120997875  SEC62; translocation protein SEC62 [KO:K12275]
120998243  SEC63; translocation protein SEC63 homolog [KO:K09540]
120978463  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
121003309  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
120989004  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
120994716  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
121000324  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
121002065  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
120978006  MAGT1; magnesium transporter protein 1 [KO:K19478]
120990185  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
120989928  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
120980345  TMEM258; transmembrane protein 258 [KO:K26502]
120990575  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
121007598  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
120998816  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
120995924  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
121009171  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
121008149  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
121002831  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
120986622  dnaJ homolog subfamily C member 3-like [KO:K09523]
120995851  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
121007840  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
120979258  HSP90B1; endoplasmin [KO:K09487]
120980125  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
120986436  PRKCSH; glucosidase 2 subunit beta [KO:K08288]
121008397  CANX; calnexin isoform X1 [KO:K08054]
120985891  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
120979853  calreticulin-like [KO:K08057]
120986427  CALR; calreticulin [KO:K08057]
120998301  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
120994322  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
120995333  mannosyl-oligosaccharide 1,2-alpha-mannosidase IA-like [KO:K01230] [EC:3.2.1.113]
120977844  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
120978591  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
120992233  LMAN1; protein ERGIC-53 [KO:K10080]
120986213  LMAN1L; protein ERGIC-53-like [KO:K10080]
120996982  PREB; prolactin regulatory element-binding protein [KO:K14003]
121009448  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
121004370  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
120979564  SEC13; protein SEC13 homolog [KO:K14004]
120989765  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
121003070  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
120998826  SEC23B; protein transport protein Sec23B [KO:K14006]
120989889  SEC24D; protein transport protein Sec24D [KO:K14007]
120989926  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
121009454  SEC24A; protein transport protein Sec24A [KO:K14007]
121003208  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
120997501  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
120995850  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
120979093  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
121003261  ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
120979716  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
121005850  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
121000998  PDIA4; LOW QUALITY PROTEIN: protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
120997393  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
120988541  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
121001160  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
120981794  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
120996077  OS9; protein OS-9 isoform X1 [KO:K10088]
120998868  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
121001156  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
120982118  SSR2; translocon-associated protein subunit beta [KO:K13250]
120997831  SSR3; translocon-associated protein subunit gamma [KO:K13251]
120977692  SSR4; translocon-associated protein subunit delta [KO:K04571]
121009541  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
121001636  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
121001297  DERL1; derlin-1 [KO:K11519]
120995579  DERL2; derlin-2 [KO:K13989]
120988732  DERL3; derlin-3 [KO:K13989]
120980785  UBXN1; LOW QUALITY PROTEIN: UBX domain-containing protein 1 [KO:K24348]
121007692  UBXN4; UBX domain-containing protein 4 [KO:K24348]
120997370  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
120989559  UBXN6; UBX domain-containing protein 6 [KO:K14011]
120989652  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
121005187  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
120980590  small VCP/p97-interacting protein [KO:K14014]
120992308  transitional endoplasmic reticulum ATPase [KO:K13525]
121004451  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
120988697  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
120997776  heat shock cognate 71 kDa protein-like [KO:K03283]
120991481  LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein-like [KO:K03283]
120999953  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
121009580  heat shock 70 kDa protein-like [KO:K03283]
121009392  heat shock 70 kDa protein-like [KO:K03283]
120989624  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
120980137  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
120986217  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
121008863  DNAJB2; dnaJ homolog subfamily B member 2 [KO:K09508]
121003734  DNAJB12; dnaJ homolog subfamily B member 12 [KO:K09518]
121006154  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
121002094  DNAJC5B; dnaJ homolog subfamily C member 5B isoform X1 [KO:K09525]
120997002  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
120999822  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
120982543  LOW QUALITY PROTEIN: heat shock protein HSP 90-alpha-like [KO:K04079]
120982544  heat shock protein HSP 90-alpha-like [KO:K04079]
120996310  HSPH1; heat shock protein 105 kDa [KO:K09485]
121001877  BAG1; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555]
120996924  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
121001317  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
120993157  CRYAA; alpha-crystallin A chain [KO:K09541]
120993791  HSPB6; heat shock protein beta-6 [KO:K09542]
120996464  CRYAB; alpha-crystallin B chain [KO:K09542]
120995064  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
120989221  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
120989233  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
120981951  UBQLN4; ubiquilin-4 [KO:K04523]
120989125  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
121001920  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
120982138  ATXN3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
120999798  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
120990444  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
120981700  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
121009037  NFE2L2; nuclear factor erythroid 2-related factor 2 [KO:K05638]
120978575  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
120996062  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
121001044  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
120978747  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
120977038  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
120990360  WFS1; wolframin [KO:K14020]
120980428  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
120994834  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
120988506  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
121008219  ERN2; serine/threonine-protein kinase/endoribonuclease IRE2 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
121004043  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
120977315  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
120998524  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
120986233  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
120991666  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
121003247  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
121002334  BAX; apoptosis regulator BAX [KO:K02159]
120995161  BAK1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
120980824  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
120980825  calpain-1 catalytic subunit-like isoform X1 [KO:K01367] [EC:3.4.22.52]
120999823  CAPN11; calpain-11 [KO:K01367] [EC:3.4.22.52]
121009330  calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
120999612  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
120977198  calpain-2 catalytic subunit-like [KO:K03853] [EC:3.4.22.53]
121003493  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 [KO:K10661] [EC:2.3.2.27]
120998186  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
120997579  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
120995571  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
120988555  ubiquitin-conjugating enzyme E2 G1-like [KO:K10575] [EC:2.3.2.23]
121008021  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
120981227  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
120977369  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
120988780  RNF185; E3 ubiquitin-protein ligase RNF185 isoform X1 [KO:K10666] [EC:2.3.2.27]
120985998  SELENOS; selenoprotein S [KO:K14025]
120981619  SEL1L; protein sel-1 homolog 1 [KO:K14026]
120980307  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
120979995  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
121007448  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
120989859  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
120986175  UBE2D4; ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
121004287  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
120979112  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
121002808  CUL1; cullin-1 isoform X1 [KO:K03347]
121009396  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
120999504  F-box only protein 2-like [KO:K10099]
120999484  F-box only protein 6-like [KO:K10100]
120999523  F-box only protein 44-like [KO:K10100]
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
bbuf00510  N-Glycan biosynthesis
bbuf03050  Proteasome
bbuf03060  Protein export
bbuf04210  Apoptosis
KO pathway
ko04141   
LinkDB

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