KEGG   PATHWAY: mlk04141
Entry
mlk04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Mustela lutreola (European mink)
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
mlk04141  Protein processing in endoplasmic reticulum
mlk04141

Other DBs
GO: 0030433 0034976
Organism
Mustela lutreola (European mink) [GN:mlk]
Gene
131838284  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
131824301  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
131812499  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
131829151  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
131824883  SEC62; translocation protein SEC62 isoform X1 [KO:K12275]
131833543  SEC63; translocation protein SEC63 homolog [KO:K09540]
131824292  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
131807714  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
131836335  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
131810415  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
131838210  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
131824454  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
131820404  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
131821398  MAGT1; magnesium transporter protein 1 [KO:K19478]
131829438  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
131840555  transmembrane protein 258-like [KO:K26502]
131813719  TMEM258; transmembrane protein 258 [KO:K26502]
131828993  transmembrane protein 258 [KO:K26502]
131840325  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
131839373  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
131808142  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
131832103  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
131836887  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
131812295  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
131824758  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
131838179  DNAJC1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
131813873  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
131827014  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
131839347  HSP90B1; endoplasmin [KO:K09487]
131813997  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
131823555  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
131832649  CANX; calnexin [KO:K08054]
131836204  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
131823596  CALR; calreticulin [KO:K08057]
131833855  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
131808979  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
131809965  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
131813066  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like isoform X1 [KO:K23741] [EC:3.2.1.209]
131813068  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like isoform X1 [KO:K23741] [EC:3.2.1.209]
131831972  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
131811255  LMAN1; protein ERGIC-53 [KO:K10080]
131835774  LMAN1L; protein ERGIC-53-like isoform X1 [KO:K10081]
131840632  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
131828873  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
131832051  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
131824220  SEC13; protein SEC13 homolog [KO:K14004]
131828543  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
131815749  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
131836498  SEC23A; protein transport protein Sec23A [KO:K14006]
131808157  SEC23B; protein transport protein Sec23B [KO:K14006]
131828699  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
131828814  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
131829399  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
131832053  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
131826881  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
131813872  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
131824166  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
131807799  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
131814376  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
131815804  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
131829908  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
131807371  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
131811585  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
131834507  TXNDC5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
131836569  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
131829044  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
131838540  OS9; protein OS-9 isoform X1 [KO:K10088]
131840121  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
131834498  SSR1; translocon-associated protein subunit alpha [KO:K13249]
131814753  SSR2; translocon-associated protein subunit beta isoform X1 [KO:K13250]
131824948  SSR3; translocon-associated protein subunit gamma [KO:K13251]
131821203  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
131822220  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
131826722  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
131828759  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
131826445  DERL1; derlin-1 [KO:K11519]
131811840  DERL3; derlin-3 [KO:K13989]
131816334  DERL2; derlin-2 isoform X1 [KO:K13989]
131826963  UBXN4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
131814021  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
131807306  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
131823331  UBXN6; UBX domain-containing protein 6 [KO:K14011]
131820527  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
131807930  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
131810476  SVIP; small VCP/p97-interacting protein [KO:K14014]
131825881  LOW QUALITY PROTEIN: small VCP/p97-interacting protein-like [KO:K14014]
131813208  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
131816142  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
131811186  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
131837691  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
131833881  heat shock cognate 71 kDa protein-like [KO:K03283]
131834338  heat shock 70 kDa protein 1-like [KO:K03283]
131834339  heat shock 70 kDa protein 1 [KO:K03283]
131834340  heat shock 70 kDa protein 1-like [KO:K03283]
131832001  heat shock cognate 71 kDa protein-like [KO:K03283]
131826746  dnaJ homolog subfamily A member 1-like [KO:K09502]
131834185  dnaJ homolog subfamily A member 1-like [KO:K09502]
131835751  dnaJ homolog subfamily A member 1-like [KO:K09502]
131834438  dnaJ homolog subfamily A member 1-like [KO:K09502]
131813232  DNAJA1; dnaJ homolog subfamily A member 1 isoform X1 [KO:K09502]
131829283  dnaJ homolog subfamily A member 1 [KO:K09502]
131824104  dnaJ homolog subfamily A member 1-like [KO:K09502]
131809885  dnaJ homolog subfamily A member 1 [KO:K09502]
131818228  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
131823649  DNAJB1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
131827422  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
131828845  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
131826771  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
131808057  DNAJC5; dnaJ homolog subfamily C member 5 isoform X1 [KO:K09525]
131808204  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
131837003  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
131834029  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
131827404  LOW QUALITY PROTEIN: heat shock protein HSP 90-alpha-like [KO:K04079]
131832553  LOW QUALITY PROTEIN: heat shock protein HSP 90-alpha-like [KO:K04079]
131827970  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
131813707  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
131812123  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
131834132  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
131817502  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
131826015  CRYAA; alpha-crystallin A chain [KO:K09541]
131835901  CRYAB; alpha-crystallin B chain [KO:K09542]
131814308  YOD1; ubiquitin thioesterase OTU1 isoform X1 [KO:K13719] [EC:3.1.2.-]
131812862  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
131812284  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
131823597  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
131812208  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
131814424  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
131821615  UBQLN2; ubiquilin-2 [KO:K04523]
131808378  ubiquilin-3-like [KO:K04523]
131824476  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
131836753  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
131810432  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
131840420  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
131836705  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
131827050  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
131839499  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
131818296  cyclic AMP-dependent transcription factor ATF-4-like [KO:K04374]
131817767  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
131838557  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
131811845  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
131815162  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
131834326  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
131826419  WFS1; wolframin [KO:K14020]
131817365  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
131821139  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
131811244  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
131816957  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
131812685  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
131833762  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
131823426  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
131828723  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
131831160  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
131832665  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
131817775  BAX; apoptosis regulator BAX [KO:K02159]
131834235  BAK1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
131815210  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
131814739  CAPN2; calpain-2 catalytic subunit isoform X1 [KO:K03853] [EC:3.4.22.53]
131834806  caspase-12-like isoform X1 [KO:K04741] [EC:3.4.22.-]
131831581  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
131833607  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
131809298  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
131816866  UBE2G1; LOW QUALITY PROTEIN: ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
131818201  ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
131825467  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
131815167  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
131811133  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
131834318  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
131835697  SELENOS; selenoprotein S [KO:K14025]
131836800  SEL1L; protein sel-1 homolog 1 [KO:K14026]
131808120  SEL1L2; protein sel-1 homolog 2 isoform X1 [KO:K14026]
131817990  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
131818464  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
131819231  STUB1; E3 ubiquitin-protein ligase CHIP isoform X1 [KO:K09561] [EC:2.3.2.27]
131828950  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
131829776  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
131832123  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
131833389  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
131839520  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X1 [KO:K03868] [EC:2.3.2.32]
131829905  CUL1; cullin-1 [KO:K03347]
131827695  S-phase kinase-associated protein 1-like [KO:K03094]
131832040  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
131809089  FBXO2; F-box only protein 2 isoform X1 [KO:K10099]
131809090  F-box only protein 6-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
mlk00510  N-Glycan biosynthesis
mlk03050  Proteasome
mlk03060  Protein export
mlk04210  Apoptosis
mlk04930  Type II diabetes mellitus
mlk05010  Alzheimer disease
mlk05012  Parkinson disease
mlk05020  Prion disease
KO pathway
ko04141   
LinkDB

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