KEGG   PATHWAY: kmr04141
Entry
kmr04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Kryptolebias marmoratus (mangrove rivulus)
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
kmr04141  Protein processing in endoplasmic reticulum
kmr04141

Other DBs
GO: 0030433 0034976
Organism
Kryptolebias marmoratus (mangrove rivulus) [GN:kmr]
Gene
108235565  sec61a1; protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
108241220  protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
108241121  sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
108243169  sec61g; protein transport protein Sec61 subunit gamma [KO:K07342]
108232900  sec62; translocation protein SEC62 [KO:K12275]
108242433  sec63; translocation protein SEC63 homolog [KO:K09540]
108241163  rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
108251000  rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
108238613  dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
108233049  ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
108240668  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
108244714  stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
108247747  dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B-like [KO:K07151] [EC:2.4.99.18]
108250575  magnesium transporter protein 1-like [KO:K19478]
108241447  tusc3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
108234829  ostc; oligosaccharyltransferase complex subunit ostc [KO:K26501]
108229868  tmem258; transmembrane protein 258 [KO:K26502]
108231066  mogs; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
108233200  ckap4; cytoskeleton-associated protein 4 [KO:K13999]
108234110  rrbp1a; ribosome-binding protein 1a isoform X1 [KO:K14000]
108240009  sil1; nucleotide exchange factor SIL1 [KO:K14001]
108249512  hyou1; hypoxia up-regulated protein 1 [KO:K09486]
108231027  hspa5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
108238790  dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517]
108229263  dnajc1; dnaJ homolog subfamily C member 1 [KO:K09521]
108239702  dnajc3a; dnaJ homolog subfamily C member 3a [KO:K09523]
108247765  dnajc3b; dnaJ homolog subfamily C member 3b [KO:K09523]
108243537  dnajc10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
108230161  hsp90b1; endoplasmin [KO:K09487]
108231381  ganab; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
108243715  prkcsh; glucosidase 2 subunit beta [KO:K08288]
108234858  glucosidase 2 subunit beta [KO:K08288]
108251406  canx; calnexin isoform X2 [KO:K08054]
108243891  pdia8; protein disulfide isomerase family A, member 8 [KO:K08056] [EC:5.3.4.1]
108245744  pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
108232876  calr3b; calreticulin 3b [KO:K08057]
108230540  calr; calreticulin [KO:K08057]
108240356  mannosyl-oligosaccharide 1,2-alpha-mannosidase IA isoform X1 [KO:K01230] [EC:3.2.1.113]
108244885  man1a1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
108247704  man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
108245625  man1b1b; mannosidase, alpha, class 1B, member 1b [KO:K23741] [EC:3.2.1.209]
108241416  man1b1a; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
108243955  lman2; vesicular integral-membrane protein VIP36 [KO:K10082]
108230871  lman1; protein ERGIC-53 [KO:K10080]
108249230  preb; prolactin regulatory element-binding protein [KO:K14003]
108242881  sar1ab; secretion associated, Ras related GTPase 1Ab [KO:K07953] [EC:3.6.5.-]
108238100  sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
108246720  sar1aa; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
108244898  sec13; protein SEC13 homolog [KO:K14004]
108249872  sec31a; protein transport protein Sec31A isoform X1 [KO:K14005]
108248968  sec31b; protein transport protein Sec31A isoform X1 [KO:K14005]
108248717  sec23a; protein transport protein Sec23A [KO:K14006]
108236412  sec23b; protein transport protein Sec23B [KO:K14006]
108249088  sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
108234874  sec24d; protein transport protein Sec24D [KO:K14007]
108242751  si:dkey-13n15.2; protein transport protein Sec24C isoform X1 [KO:K14007]
108243972  sec24b; protein transport protein Sec24B isoform X1 [KO:K14007]
108238093  sec24a; protein transport protein Sec24A isoform X1 [KO:K14007]
108239796  uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X3 [KO:K11718] [EC:2.4.1.-]
108235171  uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
108231336  edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
108232532  si:ch211-282j22.3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
108230738  edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
108248234  p4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
108243156  pdia4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
108233493  pdia6; protein disulfide-isomerase A6 isoform X1 [KO:K09584] [EC:5.3.4.1]
108244028  txndc5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
108242097  ero1a; ERO1-like protein alpha isoform X1 [KO:K10950] [EC:1.8.4.-]
108247502  ero1b; ERO1-like protein beta isoform X2 [KO:K10976] [EC:1.8.4.-]
108235735  os9; protein OS-9 isoform X1 [KO:K10088]
108233967  erlec1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
108235676  translocon-associated protein subunit alpha [KO:K13249]
108250947  ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
108248590  ssr2; translocon-associated protein subunit beta [KO:K13250]
108238113  ssr3; translocon-associated protein subunit gamma [KO:K13251]
108244573  ssr4; translocon-associated protein subunit delta [KO:K04571]
108228463  bcap31; B-cell receptor-associated protein 31 isoform X1 [KO:K14009]
108230514  tram1; translocating chain-associated membrane protein 1 [KO:K14010]
108232153  zgc:113278; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
108232250  derl1; derlin-1 [KO:K11519]
108242793  derl3; derlin-3 isoform X2 [KO:K13989]
108229144  derl2; derlin-2 isoform X1 [KO:K13989]
108238772  ubxn4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
108231303  ubxn1; UBX domain-containing protein 1 [KO:K24348]
108231796  ubxn2a; UBX domain-containing protein 2A [KO:K24349]
108230550  ubxn6; UBX domain-containing protein 6 [KO:K14011]
108246093  nsfl1c; NSFL1 cofactor p47 isoform X1 [KO:K14012]
108230017  small VCP/p97-interacting protein [KO:K14014]
108234543  transitional endoplasmic reticulum ATPase [KO:K13525]
108241508  zgc:136908 [KO:K13525]
108245584  nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
108233697  ufd1l; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
108241987  hsc70; heat shock cognate 71 kDa protein [KO:K03283]
108249515  hspa8; heat shock cognate 71 kDa protein [KO:K03283]
108243113  hspa8b; heat shock protein family A (Hsp70) member 8b [KO:K03283]
108251650  heat shock 70 kDa protein 1-like [KO:K03283]
108228473  heat shock 70 kDa protein 1-like [KO:K03283]
108251765  heat shock 70 kDa protein 1-like [KO:K03283]
108236850  hspa1b; heat shock protein family A (Hsp70) member 1B [KO:K03283]
108232675  dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502]
108241615  dnaja2a; dnaJ homolog subfamily A member 2a [KO:K09503]
108230153  dnaja2b; dnaJ homolog subfamily A member 2b [KO:K09503]
108241338  dnajb1a; dnaJ homolog subfamily B member 1a [KO:K09507]
108241484  dnajb1b; dnaJ homolog subfamily B member 1b [KO:K09507]
108240420  dnajb2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
108242887  dnajb12a; dnaJ homolog subfamily B member 12a [KO:K09518]
108246078  dnajb12b; dnaJ homolog subfamily B member 12b [KO:K09518]
108250532  dnaJ homolog subfamily C member 5 [KO:K09525]
108247046  dnajc5ga; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma a isoform X1 [KO:K09525]
108228686  dnaJ homolog subfamily C member 5 [KO:K09525]
108239103  dnajc5gb; dnaJ (Hsp40) homolog, subfamily C, member 5 gamma b isoform X1 [KO:K09525]
108232150  dnajc5aa; dnaJ (Hsp40) homolog, subfamily C, member 5aa isoform X1 [KO:K09525]
108231826  hsp90aa1.2; heat shock protein 90, alpha (cytosolic), class A member 1, tandem duplicate 2 [KO:K04079]
108233464  hsp90ab1; heat shock protein HSP 90-beta [KO:K04079]
108231827  hsp90aa1.1; heat shock protein HSP 90-alpha 1 [KO:K04079]
108232245  hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
108232978  bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
108234032  bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
108242937  hspbp1; hsp70-binding protein 1 [KO:K09562]
108241933  hspb6; heat shock protein beta-6 [KO:K09541]
108241839  cryaa; alpha-crystallin A chain [KO:K09541]
108236942  alpha-crystallin A chain-like [KO:K09542]
108231630  yod1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
108234826  plaa; phospholipase A-2-activating protein [KO:K14018]
108247520  rad23aa; RAD23 homolog A, nucleotide excision repair protein a [KO:K10839]
108230893  rad23b; UV excision repair protein RAD23 homolog B [KO:K10839]
108234502  ubiquilin-1 [KO:K04523]
108237986  ubqln4; ubiquilin-4 [KO:K04523]
108250608  ngly1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
108235003  atxn3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
108236828  ube4b; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
108247269  eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
108233445  eif2s1b; eukaryotic translation initiation factor 2 subunit 1b [KO:K03237]
108239794  nfe2l2a; nuclear factor erythroid 2-related factor 2a [KO:K05638]
108235275  nfe2; transcription factor NF-E2 45 kDa subunit [KO:K05638]
108231541  atf4a; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374]
108236059  atf4b; activating transcription factor 4b [KO:K04374]
108231469  ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
108239676  bcl2b; apoptosis regulator Bcl-2 [KO:K02161]
108229311  apoptosis regulator Bcl-2 [KO:K02161]
108230853  atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
108246380  atf6b; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
108243015  wfs1b; wolframin [KO:K14020]
108241546  wfs1a; wolframin [KO:K14020]
108246903  mbtps1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
108234732  mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
108242736  xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
108239231  serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
108230211  ern2; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
108249884  traf2a; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
108244062  TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
108230862  traf2b; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
108240373  si:ch211-1i11.3; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
108233483  map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
108242723  map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
108230987  mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
108234067  mapk8a; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
108240588  mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
108246126  apoptosis regulator BAX [KO:K02159]
108246134  apoptosis regulator BAX [KO:K02159]
108237852  zgc:153993; apoptosis regulator BAX [KO:K02159]
108247006  LOW QUALITY PROTEIN: calpain-1 catalytic subunit-like [KO:K01367] [EC:3.4.22.52]
108248456  capn1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
108234076  capn1a; calpain 1, (mu/I) large subunit a [KO:K01367] [EC:3.4.22.52]
108251140  capn1b; calpain-1 catalytic subunit b [KO:K01367] [EC:3.4.22.52]
108251143  calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
108251144  capn2b; calpain 2, (m/II) large subunit b [KO:K03853] [EC:3.4.22.53]
108234077  calpain-2 catalytic subunit-like [KO:K03853] [EC:3.4.22.53]
108234124  calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
108238666  E3 ubiquitin-protein ligase MARCHF6-like isoform X1 [KO:K10661] [EC:2.3.2.27]
108243130  marchf6; E3 ubiquitin-protein ligase MARCHF6 [KO:K10661] [EC:2.3.2.27]
108248743  ube2j1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
108239818  ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
108233867  ube2g1a; ubiquitin-conjugating enzyme E2 G1a [KO:K10575] [EC:2.3.2.23]
108235011  ube2g1b; ubiquitin-conjugating enzyme E2G 1b [KO:K10575] [EC:2.3.2.23]
108229592  ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
108250423  syvn1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
108243947  rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
108232560  rnf185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
108251565  selenos; selenoprotein S [KO:K14025]
108231894  sel1l; protein sel-1 homolog 1 [KO:K14026]
108243440  herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
108237415  E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
108237258  stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
108236573  ube2d4; ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
108242687  ubiquitin-conjugating enzyme E2 D4-like [KO:K06689] [EC:2.3.2.23]
108251675  ubiquitin-conjugating enzyme E2 D2-like isoform X2 [KO:K06689] [EC:2.3.2.23]
108228324  ubiquitin-conjugating enzyme E2 2-like [KO:K06689] [EC:2.3.2.23]
108237978  ube2d2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
108236172  rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
108249552  cullin-1 [KO:K03347]
108238075  skp1; S-phase kinase-associated protein 1 [KO:K03094]
108240314  fbxo2; F-box only protein 2 [KO:K10099]
108231943  F-box only protein 6 [KO:K10100]
108231955  F-box only protein 6 [KO:K10100]
108232069  F-box only protein 6 [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
kmr00510  N-Glycan biosynthesis
kmr03050  Proteasome
kmr03060  Protein export
kmr04210  Apoptosis
KO pathway
ko04141   
LinkDB

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