KEGG   PATHWAY: mlx04141
Entry
mlx04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Mirounga leonina (Southern elephant seal)
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
mlx04141  Protein processing in endoplasmic reticulum
mlx04141

Other DBs
GO: 0030433 0034976
Organism
Mirounga leonina (Southern elephant seal) [GN:mlx]
Gene
117997852  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
117998240  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
117998245  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
117998520  [KO:K09502]
117998640  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X1 [KO:K03868] [EC:2.3.2.32]
117998657  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
117998779  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
117998807  HSP90B1; endoplasmin [KO:K09487]
117999771  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
117999837  DERL3; derlin-3 [KO:K13989]
117999922  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
117999939  DERL2; derlin-2 isoform X1 [KO:K13989]
118000469  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
118000541  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
118000625  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
118000738  BAK1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
118000829  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
118001313  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
118001448  UBQLN2; ubiquilin-2 [KO:K04523]
118001507  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
118001568  SEC63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
118001625  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
118001822  [KO:K04556] [EC:2.3.2.31]
118001946  [KO:K04556] [EC:2.3.2.31]
118002022  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
118002128  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
118002421  SEC23A; protein transport protein Sec23A [KO:K14006]
118002428  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
118002631  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
118002645  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
118002672  ERO1A; ERO1-like protein alpha isoform X1 [KO:K10950] [EC:1.8.4.-]
118002940  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
118002982  LMAN1L; LOW QUALITY PROTEIN: protein ERGIC-53-like [KO:K10081]
118002995  [KO:K07953] [EC:3.6.5.-]
118003056  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
118003414  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
118003936  [KO:K09502]
118004154  UBQLN3; ubiquilin-3 [KO:K04523]
118004389  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
118004686  LMAN1; protein ERGIC-53 [KO:K10080]
118004893  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
118004944  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
118004982  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
118005153  PRKCSH; glucosidase 2 subunit beta [KO:K08288]
118005180  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
118005645  SSR3; translocon-associated protein subunit gamma isoform X1 [KO:K13251]
118005923  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
118005960  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
118006015  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
118006088  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
118006090  CALR; calreticulin [KO:K08057]
118006105  SEC62; translocation protein SEC62 [KO:K12275]
118006239  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
118006922  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
118007411  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
118007594  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
118007628  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
118007640  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
118007685  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
118007879  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
118007948  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
118008138  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
118008662  OS9; protein OS-9 isoform X1 [KO:K10088]
118008672  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
118009289  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
118009290  DNAJC3; dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
118009534  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
118009991  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
118010024  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
118010368  [KO:K04440] [EC:2.7.11.24]
118010389  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
118010732  WFS1; wolframin [KO:K14020]
118010862  [KO:K06689] [EC:2.3.2.23]
118011197  SEC23B; protein transport protein Sec23B [KO:K14006]
118011212  RRBP1; LOW QUALITY PROTEIN: ribosome-binding protein 1 [KO:K14000]
118011224  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
118011375  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
118011413  [KO:K10085]
118011459  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
118012020  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
118012253  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
118012291  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
118012499  UBXN4; UBX domain-containing protein 4 [KO:K24348]
118012583  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
118012936  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
118012992  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
118013166  [KO:K04741] [EC:3.4.22.-]
118013225  CRYAB; alpha-crystallin B chain [KO:K09542]
118013301  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
118013335  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
118013369  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
118013453  [KO:K14012]
118013736  [KO:K06689] [EC:2.3.2.23]
118013784  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
118013876  KRTCAP2; keratinocyte-associated protein 2 isoform X1 [KO:K28202]
118013895  SSR2; translocon-associated protein subunit beta [KO:K13250]
118013898  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
118014029  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
118014257  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
118014386  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
118014880  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
118014908  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
118014909  TRAM1; translocating chain-associated membrane protein 1 isoform X1 [KO:K14010]
118014922  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
118014942  [KO:K04079]
118015172  DERL1; derlin-1 [KO:K11519]
118015698  SVIP; small VCP/p97-interacting protein [KO:K14014]
118015899  [KO:K07342]
118015926  TMEM258; transmembrane protein 258 [KO:K26502]
118015944  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
118015948  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
118016032  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
118016038  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
118016639  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
118016649  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
118016650  SEC24A; protein transport protein Sec24A [KO:K14007]
118016691  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
118016703  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
118017286  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
118017503  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
118017681  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
118017848  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
118017898  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
118017985  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
118018031  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
118018056  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
118018152  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
118018322  [KO:K07953] [EC:3.6.5.-]
118018482  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
118018617  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
118018666  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
118018867  [KO:K24349]
118019045  [KO:K07953] [EC:3.6.5.-]
118019407  [KO:K06689] [EC:2.3.2.23]
118019859  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
118019882  [KO:K06689] [EC:2.3.2.23]
118019926  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
118020003  FBXO6; F-box only protein 6 [KO:K10100]
118020021  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
118020131  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
118020340  FBXO2; F-box only protein 2 [KO:K10099]
118020350  [KO:K24349]
118020648  [KO:K03283]
118020674  [KO:K09502]
118021211  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
118021218  CUL1; cullin-1 [KO:K03347]
118021222  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
118021514  DNAJC1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
118021582  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
118021640  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
118021668  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
118022334  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
118022776  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
118022986  SELENOS; selenoprotein S [KO:K14025]
118023149  [KO:K13251]
118023307  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
118023513  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
118023758  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
118023769  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
118023822  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
118024065  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
118024165  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
118024172  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
118024372  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
118024534  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
118024939  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
118026048  MAGT1; magnesium transporter protein 1 [KO:K19478]
118026506  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
118026514  CANX; calnexin isoform X1 [KO:K08054]
118026918  CRYAA; alpha-crystallin A chain [KO:K09541]
118026950  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
118027026  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
118027049  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
118027082  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
118027085  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
118027119  SEC24D; protein transport protein Sec24D [KO:K14007]
118027303  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
118027308  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
118027322  HSPA1L; heat shock 70 kDa protein 1-like isoform X1 [KO:K03283]
118027433  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
118027441  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
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
mlx00510  N-Glycan biosynthesis
mlx03050  Proteasome
mlx03060  Protein export
mlx04210  Apoptosis
mlx04930  Type II diabetes mellitus
mlx05010  Alzheimer disease
mlx05012  Parkinson disease
mlx05020  Prion disease
KO pathway
ko04141   

DBGET integrated database retrieval system