KEGG   PATHWAY: bbis04141
Entry
bbis04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Bison bison bison (American bison)
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
bbis04141  Protein processing in endoplasmic reticulum
bbis04141

Other DBs
GO: 0030433 0034976
Organism
Bison bison bison (American bison) [GN:bbis]
Gene
104979690  UBXN6; UBX domain-containing protein 6 [KO:K14011]
104979935  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
104979953  PRKCSH; glucosidase 2 subunit beta [KO:K08288]
104980039  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
104980158  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
104980220  XBP1; X-box-binding protein 1 [KO:K09027]
104980469  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
104980690  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
104980886  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
104981072  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
104981132  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
104981171  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
104981421  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
104981504  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
104981526  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
104981535  CANX; calnexin [KO:K08054]
104981559  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
104981975  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
104982185  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
104982187  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
104982944  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
104983375  SEC61A1; protein transport protein Sec61 subunit alpha isoform 1 [KO:K10956]
104983797  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
104983839  [KO:K12666]
104983968  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
104983973  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
104984118  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
104984195  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
104984275  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
104984424  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
104984731  ERO1L; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
104984779  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
104984918  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
104984939  CRYAA; alpha-crystallin A chain [KO:K09541]
104985183  SEC62; translocation protein SEC62 [KO:K12275]
104985255  SEC23A; protein transport protein Sec23A [KO:K14006]
104985404  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
104985602  SSR1; translocon-associated protein subunit alpha [KO:K13249]
104985640  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
104985656  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
104985794  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
104985815  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
104985939  SEC23B; protein transport protein Sec23B [KO:K14006]
104986022  UFD1L; ubiquitin fusion degradation protein 1 homolog [KO:K14016]
104986107  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
104986252  PARK2; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
104987110  [KO:K09584] [EC:5.3.4.1]
104987162  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
104987219  SYVN1; LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
104987221  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
104987384  SSR2; translocon-associated protein subunit beta isoform X1 [KO:K13250]
104987410  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
104987476  UBQLN4; ubiquilin-4 [KO:K04523]
104987749  UBXN8; UBX domain-containing protein 8 [KO:K24351]
104987776  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
104987846  DERL1; derlin-1 [KO:K11519]
104988119  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
104988131  OS9; protein OS-9 isoform X1 [KO:K10088]
104988357  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
104988523  SSR3; translocon-associated protein subunit gamma [KO:K13251]
104988673  UBQLN1; ubiquilin-1 [KO:K04523]
104988706  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
104988713  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
104989022  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
104989070  PREB; prolactin regulatory element-binding protein [KO:K14003]
104989076  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
104989876  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
104989945  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
104990112  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
104990704  UBQLN2; ubiquilin-2 [KO:K04523]
104990987  LMAN1L; protein ERGIC-53-like [KO:K10081]
104991218  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
104991415  CUL1; cullin-1 [KO:K03347]
104991425  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
104991438  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
104991546  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
104991592  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
104991606  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
104991715  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
104991852  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
104991855  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
104991862  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
104992826  VIMP; selenoprotein S isoform X1 [KO:K14025]
104992919  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
104992939  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
104993030  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
104993271  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
104993337  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
104993460  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
104993511  MAGT1; magnesium transporter protein 1 [KO:K19478]
104993649  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
104993679  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
104993682  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
104993718  DERL3; derlin-3 isoform X1 [KO:K13989]
104993979  DERL2; derlin-2 isoform X1 [KO:K13989]
104994004  UBE2G1; ubiquitin-conjugating enzyme E2 G1 isoform X1 [KO:K10575] [EC:2.3.2.23]
104994084  MARCH6; E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
104994153  [KO:K11863] [EC:3.4.22.-]
104994281  WFS1; wolframin [KO:K14020]
104994634  [KO:K06689] [EC:2.3.2.23]
104994635  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
104994813  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
104994860  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
104994906  BAK1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
104995628  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
104995631  BAX; apoptosis regulator BAX [KO:K02159]
104995842  FBXO6; F-box only protein 6 [KO:K10100]
104995859  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
104996051  FBXO2; F-box only protein 2 [KO:K10099]
104996100  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
104996178  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
104996192  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
104996344  [KO:K09481]
104996529  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
104996599  SEL1L; protein sel-1 homolog 1 [KO:K14026]
104996944  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
104997399  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
104997467  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
104997488  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
104997511  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
104997873  [KO:K03853] [EC:3.4.22.53]
104998072  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
104998251  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
104998313  HSP90B1; endoplasmin [KO:K09487]
104998347  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
104998401  HSPBP1; LOW QUALITY PROTEIN: hsp70-binding protein 1 [KO:K09562]
104998485  UBXN4; UBX domain-containing protein 4 [KO:K24348]
104998623  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
104998685  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
104998709  TMEM258; transmembrane protein 258 [KO:K26502]
104998724  GANAB; neutral alpha-glucosidase AB [KO:K05546] [EC:3.2.1.207]
104998764  SEC61A2; protein transport protein Sec61 subunit alpha isoform 2 isoform X1 [KO:K10956]
104998835  YOD1; ubiquitin thioesterase OTU1 isoform X1 [KO:K13719] [EC:3.1.2.-]
104998999  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
104999179  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
104999187  HSPA6; heat shock 70 kDa protein 6 [KO:K03283]
104999231  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
104999284  LMAN1; protein ERGIC-53 [KO:K10080]
104999340  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
104999670  SEC24D; protein transport protein Sec24D [KO:K14007]
104999676  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
104999817  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
104999841  CALR; calreticulin [KO:K08057]
104999902  RAD23A; UV excision repair protein RAD23 homolog A [KO:K10839]
105000429  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
105000438  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
105000492  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
105000627  SSR4; translocon-associated protein subunit delta [KO:K04571]
105000709  SEC63; translocation protein SEC63 homolog [KO:K09540]
105001314  [KO:K06689] [EC:2.3.2.23]
105002082  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
105002088  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
105002713  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
105002927  NFE2L2; nuclear factor erythroid 2-related factor 2 [KO:K05638]
105003089  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
105003148  ERO1LB; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
105003221  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
105003294  UBQLN3; ubiquilin-3 [KO:K04523]
105003603  [KO:K10839]
105003864  DNAJB2; dnaJ homolog subfamily B member 2 [KO:K09508]
105004314  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
105004323  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
105004364  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
105004723  SEC13; protein SEC13 homolog [KO:K14004]
105004968  RRBP1; ribosome-binding protein 1 [KO:K14000]
105005031  CRYAB; alpha-crystallin B chain [KO:K09542]
105005197  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
105005565  [KO:K06689] [EC:2.3.2.23]
105005597  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
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
bbis00510  N-Glycan biosynthesis
bbis03050  Proteasome
bbis03060  Protein export
bbis04210  Apoptosis
bbis04930  Type II diabetes mellitus
bbis05010  Alzheimer disease
bbis05012  Parkinson disease
bbis05020  Prion disease
KO pathway
ko04141   

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