KEGG   PATHWAY: etf04141
Entry
etf04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Echinops telfairi (small Madagascar hedgehog)
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
etf04141  Protein processing in endoplasmic reticulum
etf04141

Other DBs
GO: 0030433 0034976
Organism
Echinops telfairi (small Madagascar hedgehog) [GN:etf]
Gene
101646245  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
101657341  SEC61A2; protein transport protein Sec61 subunit alpha isoform X2 [KO:K10956]
101657133  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
115871880  protein transport protein Sec61 subunit gamma-like [KO:K07342]
115868143  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
115869230  LOW QUALITY PROTEIN: protein transport protein Sec61 subunit gamma-like [KO:K07342]
101658660  SEC62; translocation protein SEC62 [KO:K12275]
101653331  SEC63; translocation protein SEC63 homolog [KO:K09540]
101648147  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
101644726  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
101660196  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
101643815  DDOST; LOW QUALITY PROTEIN: dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
101661719  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
101657463  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
101658153  magnesium transporter protein 1-like [KO:K19478]
101646325  MAGT1; magnesium transporter protein 1 [KO:K19478]
101664370  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
101646568  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
101650190  TMEM258; transmembrane protein 258 isoform X2 [KO:K26502]
101643012  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
101646447  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
101639050  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
101641869  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
101651816  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
101655317  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
101650902  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
101663009  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
101643239  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
101648243  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
101656459  LOW QUALITY PROTEIN: endoplasmin-like [KO:K09487]
101642841  HSP90B1; endoplasmin [KO:K09487]
101650593  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
101642949  PRKCSH; LOW QUALITY PROTEIN: glucosidase 2 subunit beta [KO:K08288]
101656938  CANX; calnexin [KO:K08054]
101644593  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
101661269  calreticulin-like [KO:K08057]
101651535  CALR; calreticulin [KO:K08057]
101655239  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
101644980  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
101662395  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
101652943  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like [KO:K23741] [EC:3.2.1.209]
101639164  MAN1B1; LOW QUALITY PROTEIN: endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
101645345  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
101657503  LMAN1; protein ERGIC-53 [KO:K10080]
101661855  LMAN1L; protein ERGIC-53-like [KO:K10081]
101651600  PREB; prolactin regulatory element-binding protein isoform X2 [KO:K14003]
101658284  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
101662222  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
101638996  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
101650646  SEC31A; protein transport protein Sec31A isoform X8 [KO:K14005]
101649980  SEC23B; protein transport protein Sec23B [KO:K14006]
101660735  SEC23A; protein transport protein Sec23A [KO:K14006]
101658602  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
101647524  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
101648006  SEC24D; protein transport protein Sec24D [KO:K14007]
101644334  SEC24C; protein transport protein Sec24C isoform X2 [KO:K14007]
101649282  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
101656422  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
101653824  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
101657879  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
101657839  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X2 [KO:K10086]
101663223  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
101661566  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
101654129  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
101651906  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
101654961  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
101647173  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
101644811  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
101647185  OS9; protein OS-9 isoform X3 [KO:K10088]
101651799  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
101663234  SSR1; translocon-associated protein subunit alpha isoform X3 [KO:K13249]
101642727  SSR2; translocon-associated protein subunit beta [KO:K13250]
101646359  SSR3; translocon-associated protein subunit gamma [KO:K13251]
101642294  translocon-associated protein subunit delta-like [KO:K04571]
101664001  SSR4; translocon-associated protein subunit delta [KO:K04571]
101663300  B-cell receptor-associated protein 31-like [KO:K14009]
101655844  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
101645806  TRAM1L1; LOW QUALITY PROTEIN: translocating chain-associated membrane protein 1-like 1 [KO:K14010]
101648811  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
101657557  DERL1; derlin-1 [KO:K11519]
101641902  DERL2; derlin-2 isoform X1 [KO:K13989]
101638129  DERL3; derlin-3 [KO:K13989]
101651519  UBXN1; UBX domain-containing protein 1 [KO:K24348]
101660458  UBXN4; UBX domain-containing protein 4 [KO:K24348]
101642708  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
101641882  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
101643854  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
101638217  NSFL1C; NSFL1 cofactor p47 isoform X2 [KO:K14012]
115869874  small VCP/p97-interacting protein [KO:K14014]
101651199  transitional endoplasmic reticulum ATPase [KO:K13525]
101648986  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
101654483  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X2 [KO:K14016]
101660675  LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein-like [KO:K03283]
101659653  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
101660593  HSPA1B; heat shock 70 kDa protein 1B [KO:K03283]
101639818  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
101664300  LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 1-like [KO:K09502]
101664478  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
101642868  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
101661651  dnaJ homolog subfamily B member 1-like [KO:K09507]
101649989  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
101663806  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
101638988  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
101654743  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
101656441  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
101655291  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
101649746  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
101650153  LOW QUALITY PROTEIN: heat shock protein HSP 90-beta-like [KO:K04079]
101639274  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
101658530  HSPH1; heat shock protein 105 kDa [KO:K09485]
101644173  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
101642905  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
101654334  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
101655092  HSPBP1; hsp70-binding protein 1 isoform X2 [KO:K09562]
101647981  CRYAA; alpha-crystallin A chain [KO:K09541]
101644795  CRYAB; alpha-crystallin B chain [KO:K09542]
101649253  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
101638509  PLAA; phospholipase A-2-activating protein [KO:K14018]
101641757  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
101650813  RAD23A; UV excision repair protein RAD23 homolog A isoform X3 [KO:K10839]
101643357  UBQLN4; ubiquilin-4 [KO:K04523]
101658238  UBQLN2; ubiquilin-2 [KO:K04523]
101650405  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
101643900  UBQLN3; ubiquilin-3 [KO:K04523]
101641726  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
101648219  ATXN3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
101639738  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
101641434  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
101639489  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
101660660  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X2 [KO:K05638]
101650311  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
101652164  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
101659722  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
101659622  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
101662802  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X2 [KO:K09049]
101662197  WFS1; wolframin [KO:K14020]
101641729  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
101646070  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
101654999  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
101660158  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
101643556  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
101645193  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
101663385  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
101654493  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
101653598  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
101645489  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
101646711  BAX; apoptosis regulator BAX [KO:K02159]
101656097  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
101659155  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
101648937  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
101653369  caspase-12 [KO:K04741] [EC:3.4.22.-]
101642820  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 [KO:K10661] [EC:2.3.2.27]
101660284  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
101654291  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
101660112  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
101642914  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
101664492  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X3 [KO:K10601] [EC:2.3.2.27]
101638400  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
101647606  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
101663283  SELENOS; selenoprotein S [KO:K14025]
101642098  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
101651016  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
101651268  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
101659835  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
101650969  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
101658935  ubiquitin-conjugating enzyme E2 D2-like [KO:K06689] [EC:2.3.2.23]
101657657  ubiquitin-conjugating enzyme E2 D2-like [KO:K06689] [EC:2.3.2.23]
101646552  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
101662972  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
101638777  ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
101643973  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
101639304  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
101662489  E3 ubiquitin-protein ligase RBX1-like [KO:K03868] [EC:2.3.2.32]
101646762  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
101648516  CUL1; cullin-1 isoform X3 [KO:K03347]
101648514  S-phase kinase-associated protein 1-like [KO:K03094]
105978575  S-phase kinase-associated protein 1 [KO:K03094]
101663453  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
101639322  FBXO2; F-box only protein 2 [KO:K10099]
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
etf00510  N-Glycan biosynthesis
etf03050  Proteasome
etf03060  Protein export
etf04210  Apoptosis
etf04930  Type II diabetes mellitus
etf05010  Alzheimer disease
etf05012  Parkinson disease
etf05020  Prion disease
KO pathway
ko04141   
LinkDB

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