KEGG   PATHWAY: btax04141
Entry
btax04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Budorcas taxicolor (takin)
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
btax04141  Protein processing in endoplasmic reticulum
btax04141

Other DBs
GO: 0030433 0034976
Organism
Budorcas taxicolor (takin) [GN:btax]
Gene
128043232  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
128043265  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
128044369  [KO:K19478]
128044370  HSPA6; heat shock 70 kDa protein 6 [KO:K03283]
128044397  UBQLN4; ubiquilin-4 [KO:K04523]
128044733  [KO:K09525]
128044797  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
128044887  SSR2; translocon-associated protein subunit beta [KO:K13250]
128044923  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
128045138  KRTCAP2; LOW QUALITY PROTEIN: keratinocyte-associated protein 2 [KO:K28202]
128045159  SSR3; translocon-associated protein subunit gamma [KO:K13251]
128046241  CUL1; cullin-1 [KO:K03347]
128046448  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
128046705  [KO:K03094]
128046932  [KO:K03347]
128047244  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
128047271  OS9; protein OS-9 isoform X1 [KO:K10088]
128047285  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
128047331  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
128047416  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
128047442  HSP90B1; endoplasmin [KO:K09487]
128047495  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X1 [KO:K03868] [EC:2.3.2.32]
128047504  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
128047646  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
128047733  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
128047755  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
128047811  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
128047885  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
128048231  [KO:K24349]
128048350  [KO:K04374]
128048589  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
128048850  SEC62; translocation protein SEC62 [KO:K12275]
128049402  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
128049576  WFS1; wolframin isoform X1 [KO:K14020]
128049619  MAPK10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
128049762  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
128049902  [KO:K14025]
128049960  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
128049975  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
128050043  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
128050074  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
128050089  SEC24D; protein transport protein Sec24D [KO:K14007]
128050226  CRYAA; alpha-crystallin A chain [KO:K09541]
128050263  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
128050272  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
128050281  SEC24A; protein transport protein Sec24A [KO:K14007]
128050282  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
128050643  CANX; calnexin [KO:K08054]
128050647  LMAN2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
128050697  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
128050748  SEC61G; protein transport protein Sec61 subunit gamma isoform X1 [KO:K07342]
128050765  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
128050843  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
128050932  CALR; calreticulin [KO:K08057]
128050933  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
128050937  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
128051495  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
128051602  DNAJB1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
128052032  UBQLN1; ubiquilin-1 [KO:K04523]
128052047  [KO:K09054]
128052084  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
128052137  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
128052220  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
128052242  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
128052368  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
128052495  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
128052564  [KO:K12666]
128053033  SEC63; translocation protein SEC63 homolog [KO:K09540]
128053056  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
128053059  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
128053214  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
128053318  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
128054014  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
128054015  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
128054079  [KO:K09502]
128054117  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
128054174  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
128055080  [KO:K03283]
128055112  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
128055123  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
128055290  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
128055295  [KO:K03283]
128055316  PREB; prolactin regulatory element-binding protein [KO:K14003]
128055366  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
128055367  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
128055475  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
128055766  [KO:K23741]
128055818  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
128055898  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
128055959  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
128056082  BAK1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
128056122  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
128056128  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
128056196  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
128056429  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
128056658  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
128056836  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
128057496  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
128057554  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
128057624  [KO:K11718] [EC:2.4.1.-]
128057773  [KO:K11718] [EC:2.4.1.-]
128057775  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
128057776  [KO:K09523]
128058145  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
128058187  SEC23B; protein transport protein Sec23B [KO:K14006]
128058227  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
128058332  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
128058336  [KO:K03094]
128058370  RRBP1; ribosome-binding protein 1 [KO:K14000]
128058475  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
128058536  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
128058626  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
128058635  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
128059160  DERL1; derlin-1 [KO:K11519]
128059240  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
128059321  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
128060331  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
128060871  UBQLN3; LOW QUALITY PROTEIN: ubiquilin-3 [KO:K04523]
128061001  CRYAB; alpha-crystallin B chain [KO:K09542]
128061031  HSPA8; heat shock cognate 71 kDa protein isoform X1 [KO:K03283]
128061180  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
128061211  UBE4B; ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
128061280  UBE2D4; ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
128061410  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
128061453  FBXO6; F-box only protein 6 [KO:K10100]
128061596  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
128061828  FBXO2; F-box only protein 2 [KO:K10099]
128061856  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
128062235  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
128062290  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
128062328  DERL3; derlin-3 isoform X1 [KO:K13989]
128062362  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
128062446  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
128062500  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
128062881  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
128062939  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
128063013  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
128063110  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
128063211  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
128063229  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
128063406  [KO:K09584] [EC:5.3.4.1]
128063560  HSPBP1; hsp70-binding protein 1 [KO:K09562]
128064207  BAX; apoptosis regulator BAX [KO:K02159]
128064383  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
128064598  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
128064658  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
128064682  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
128064719  STUB1; E3 ubiquitin-protein ligase CHIP isoform X1 [KO:K09561] [EC:2.3.2.27]
128064885  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
128064924  DERL2; derlin-2 isoform X1 [KO:K13989]
128065162  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
128065507  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
128066042  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
128066272  [KO:K03094]
128066397  SEC23A; protein transport protein Sec23A isoform X1 [KO:K14006]
128066470  LMAN1L; protein ERGIC-53-like [KO:K10081]
128066552  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
128066717  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
128066741  ATXN3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
128066754  SELENOS; selenoprotein S [KO:K14025]
128067059  UBXN4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
128067342  LMAN1; protein ERGIC-53 [KO:K10080]
128067358  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
128067535  [KO:K03868] [EC:2.3.2.32]
128067660  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
128067711  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
128068044  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
128068240  TUSC3; tumor suppressor candidate 3 [KO:K19478]
128068285  [KO:K24349]
128068288  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
128068309  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
128068479  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
128068480  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
128068657  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
128068788  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
128068920  SVIP; small VCP/p97-interacting protein [KO:K14014]
128069011  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
128069036  TMEM258; transmembrane protein 258 [KO:K26502]
128069183  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
128069776  UBQLN2; ubiquilin-2 [KO:K04523]
128069834  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
128069876  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
128069897  MAGT1; magnesium transporter protein 1 [KO:K19478]
128069987  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
128070052  [KO:K10575] [EC:2.3.2.23]
128071265  [KO:K14003]
128071294  [KO:K24349]
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
btax00510  N-Glycan biosynthesis
btax03050  Proteasome
btax03060  Protein export
btax04210  Apoptosis
btax04930  Type II diabetes mellitus
btax05010  Alzheimer disease
btax05012  Parkinson disease
btax05020  Prion disease
KO pathway
ko04141   

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