KEGG   PATHWAY: mcep04141
Entry
mcep04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Mugil cephalus (flathead mullet)
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
mcep04141  Protein processing in endoplasmic reticulum
mcep04141

Other DBs
GO: 0030433 0034976
Organism
Mugil cephalus (flathead mullet) [GN:mcep]
Gene
124995429  pdia4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
124995573  ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
124995681  txndc5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
124995790  plaA; phospholipase A-2-activating protein [KO:K14018]
124995793  [KO:K10661] [EC:2.3.2.27]
124995796  tram1; translocating chain-associated membrane protein 1 [KO:K14010]
124995897  mbtps2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
124996345  rnf185; E3 ubiquitin-protein ligase RNF185 isoform X1 [KO:K10666] [EC:2.3.2.27]
124996389  hspa5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
124996426  traf2b; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
124996435  [KO:K10575] [EC:2.3.2.23]
124996511  derl2; derlin-2 [KO:K13989]
124996615  sec31a; protein transport protein Sec31A isoform X1 [KO:K14005]
124996962  lman1; protein ERGIC-53 [KO:K10080]
124996967  [KO:K10086]
124996988  rad23b; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
124997015  man1b1a; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741]
124997018  mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
124997163  [KO:K03173] [EC:2.3.2.27]
124997348  rad23aa; RAD23 homolog A, nucleotide excision repair protein A [KO:K10839]
124997474  [KO:K02159]
124997577  [KO:K09561] [EC:2.3.2.27]
124997628  prkcsh; glucosidase 2 subunit beta [KO:K08288]
124997994  [KO:K08852] [EC:2.7.11.1 3.1.26.-]
124998068  atf4b; activating transcription factor 4b [KO:K04374]
124998272  dnajb1a; DnaJ homolog subfamily B member 1a [KO:K09507]
124998280  [KO:K03283]
124998282  [KO:K03283]
124998308  rbx1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
124998416  hspbp1; hsp70-binding protein 1 [KO:K09562]
124998708  hsp90aa1.2; heat shock protein 90, alpha (cytosolic), class A member 1, tandem duplicate 2 [KO:K04079]
124998709  hsp90aa1.1; heat shock protein HSP 90-alpha 1 [KO:K04079]
124998721  ubxn2a; UBX domain-containing protein 2A isoform X1 [KO:K24349]
124998772  hsp90ab1; heat shock protein HSP 90-beta isoform X1 [KO:K04079]
124998774  eif2s1b; eukaryotic translation initiation factor 2 subunit 1b [KO:K03237]
124999048  pdia6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
124999094  sel1l; protein sel-1 homolog 1 [KO:K14026]
124999169  ube2j1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
124999219  sec63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
124999820  ckap4; LOW QUALITY PROTEIN: cytoskeleton-associated protein 4 [KO:K13999]
124999953  zgc:136908; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
125000964  cryaa; alpha-crystallin A chain [KO:K09541]
125001014  wfs1a; wolframin [KO:K14020]
125003493  dnaja1; DnaJ homolog subfamily A member 1 [KO:K09502]
125003557  ero1b; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
125003859  atf4a; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374]
125003915  sec23b; protein transport protein Sec23B [KO:K14006]
125003975  map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
125004162  ern2; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
125004282  p4hb; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
125004379  mogS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
125004579  capn8; calpain-8 isoform X1 [KO:K03853] [EC:3.4.22.53]
125004580  capn2l; calpain 2, (m/II) large subunit, like [KO:K03853] [EC:3.4.22.53]
125004581  capn2b; calpain 2, (m/II) large subunit B [KO:K03853] [EC:3.4.22.53]
125004585  ube4b; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
125004624  dnajb1b; DnaJ homolog subfamily B member 1b [KO:K09507]
125004789  tusc3; tumor suppressor candidate 3 [KO:K19478]
125004907  [KO:K10636] [EC:2.3.2.36]
125004974  nfe2; transcription factor NF-E2 45 kDa subunit [KO:K05638]
125005165  ostC; oligosaccharyltransferase complex subunit ostc [KO:K26501]
125005193  [KO:K14025]
125005501  sec24d; protein transport protein Sec24D [KO:K14007]
125005858  ero1a; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
125005871  dnaja2a; DnaJ homolog subfamily A member 2a [KO:K09503]
125005918  zgc:153993; apoptosis regulator BAX isoform X1 [KO:K02159]
125006406  ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
125006417  os9; protein OS-9 isoform X1 [KO:K10088]
125006534  yod1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
125006771  rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
125006818  [KO:K10956]
125006881  [KO:K13249]
125006890  sec61a1; protein transport protein Sec61 subunit alpha-like 1 [KO:K10956]
125006925  fbxo2; F-box only protein 2 [KO:K10099]
125007074  ube2j2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
125007150  edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
125007175  sec13; protein SEC13 homolog [KO:K14004]
125007274  rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
125007806  [KO:K06689] [EC:2.3.2.23]
125007861  ube2d2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
125007865  canX; calnexin [KO:K08054]
125007930  wfs1b; wolframin [KO:K14020]
125007962  ubxn1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
125007981  sil1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
125008024  ganabb; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
125008153  syvn1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
125008214  [KO:K19478]
125008348  sec24b; protein transport protein Sec24B isoform X1 [KO:K14007]
125008374  lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
125008712  mapk9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
125009077  edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
125009128  ubxn6; UBX domain-containing protein 6 [KO:K14011]
125009129  edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
125009358  calR; calreticulin [KO:K08057]
125009761  calr3a; calreticulin 3a [KO:K08057]
125009943  bcap31; B-cell receptor-associated protein 31 [KO:K14009]
125009976  atf6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
125009978  rad23ab; RAD23 homolog A, nucleotide excision repair protein B [KO:K10839]
125010354  calr3b; calreticulin 3b [KO:K08057]
125010371  [KO:K10661] [EC:2.3.2.27]
125010380  [KO:K09525]
125010408  bcl2b; apoptosis regulator Bcl-2 [KO:K02161]
125010412  sec62; translocation protein SEC62 [KO:K12275]
125010770  bag1; BAG family molecular chaperone regulator 1 [KO:K09555]
125010959  dnajc1; DnaJ homolog subfamily C member 1 [KO:K09521]
125011137  dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
125012035  [KO:K04523]
125012047  ube2d4; ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:2.3.2.23]
125012059  [KO:K13525]
125012078  man1b1b; mannosidase, alpha, class 1B, member 1b [KO:K23741]
125012098  ufd1l; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
125012326  xbp1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
125012453  [KO:K10575] [EC:2.3.2.23]
125012733  [KO:K03173] [EC:2.3.2.27]
125012878  si:dkey-13n15.2; protein transport protein Sec24C [KO:K14007]
125012903  [KO:K13989]
125012984  ssr3; translocon-associated protein subunit gamma [KO:K13251]
125013146  ssr4; translocon-associated protein subunit delta [KO:K04571]
125013395  hspa8b; heat shock protein family A (Hsp70) member 8b [KO:K03283]
125014301  hsp90b1; endoplasmin [KO:K09487]
125014744  [KO:K14014]
125014764  dnaja2b; DnaJ homolog subfamily A member 2b [KO:K09503]
125015001  mbtps1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
125015154  pdia3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
125015316  herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
125015740  dnajc3b; DnaJ homolog subfamily C member 3b [KO:K09523]
125015750  hspb6; heat shock protein beta-6 isoform X1 [KO:K09542]
125015849  [KO:K07151] [EC:2.4.99.18]
125015956  derl1; derlin-1 [KO:K11519]
125016095  hsc70; heat shock cognate 71 kDa protein [KO:K03283]
125016285  sec61b; protein transport protein Sec61 subunit beta [KO:K09481]
125016376  krtcap2; keratinocyte-associated protein 2 [KO:K28202]
125016712  [KO:K04523]
125016770  [KO:K01230] [EC:3.2.1.113]
125016978  si:ch211-1i11.3; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
125017031  preB; prolactin regulatory element-binding protein [KO:K14003]
125017269  nfe2l2a; nuclear factor erythroid 2-related factor 2a isoform X1 [KO:K05638]
125017338  dnajb2; DnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
125017424  [KO:K24348]
125017489  man1a2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
125017491  uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
125017492  dnajc3a; DnaJ homolog subfamily C member 3a [KO:K09523]
125017518  [KO:K24348]
125017616  dnajb11; DnaJ homolog subfamily B member 11 [KO:K09517]
125017985  dnajc10; DnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
125018016  zgc:113278; translocating chain-associated membrane protein 1-like 1-like [KO:K14010]
125018092  ube2g2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
125018645  rrbp1a; ribosome-binding protein 1a isoform X1 [KO:K14000]
125018735  sar1ab; secretion associated, Ras related GTPase 1Ab [KO:K07953] [EC:3.6.5.-]
125018834  capn1a; calpain 1, (mu/I) large subunit A [KO:K01367] [EC:3.4.22.52]
125018858  bag2; BAG family molecular chaperone regulator 2 [KO:K09556]
125018888  prkN; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
125018903  dnajb12a; DnaJ homolog subfamily B member 12a [KO:K09518]
125019146  [KO:K03853] [EC:3.4.22.53]
125019147  [KO:K03853] [EC:3.4.22.53]
125019216  [KO:K06689] [EC:2.3.2.23]
125019264  erlec1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
125019343  mapk8a; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
125019462  [KO:K01456] [EC:3.5.1.52]
125019463  [KO:K01456] [EC:3.5.1.52]
125019581  [KO:K04523]
125019672  ssr2; translocon-associated protein subunit beta [KO:K13250]
125019929  capn1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
125020030  [KO:K07151] [EC:2.4.99.18]
125020360  nsfl1c; NSFL1 cofactor P47 [KO:K14012]
125020377  rnf5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
125020392  atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
125020540  pdia8; protein disulfide isomerase family A, member 8 [KO:K08056] [EC:5.3.4.1]
125020625  [KO:K08288]
125020749  [KO:K01367] [EC:3.4.22.52]
125020785  [KO:K03283]
125020786  hspa8; heat shock cognate 71 kDa protein [KO:K03283]
125020800  stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
125020944  skp1; S-phase kinase-associated protein 1 [KO:K03094]
125020985  [KO:K10100]
125020995  [KO:K10100]
125021111  sar1b; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
125021117  dnajc5ab; DnaJ homolog subfamily C member 5 [KO:K09525]
125021136  [KO:K01367] [EC:3.4.22.52]
125021143  sec24a; protein transport protein Sec24A isoform X1 [KO:K14007]
125021190  hyou1; hypoxia up-regulated protein 1 [KO:K09486]
125021294  cryabb; crystallin, alpha B, B [KO:K09542]
125021757  [KO:K02159]
125022007  dnajc5aa; DnaJ (Hsp40) homolog, subfamily C, member 5aa [KO:K09525]
125022094  stub1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
125022120  ube2d1b; ubiquitin-conjugating enzyme E2 D1b [KO:K06689] [EC:2.3.2.23]
125022122  mapk8b; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
125022452  dnajb12b; DnaJ homolog subfamily B member 12b [KO:K09518]
125022460  sar1aa; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
125022468  sec24c; protein transport protein Sec24C isoform X1 [KO:K14007]
125022637  sec31b; protein transport protein Sec31A isoform X1 [KO:K14005]
125022665  nploc4; nuclear protein localization protein 4 homolog [KO:K14015]
125023208  hspa4l; heat shock 70 kDa protein 4L [KO:K09485]
125023257  dnajc5gb; DnaJ (Hsp40) homolog, subfamily C, member 5 gamma b isoform X1 [KO:K09525]
125023421  hspa1b; heat shock 70 kDa protein-like [KO:K03283]
125023509  uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
125023690  sec23a; protein transport protein Sec23A [KO:K14006]
125023797  eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
125023822  [KO:K06689] [EC:2.3.2.23]
125024190  [KO:K01367] [EC:3.4.22.52]
125024213  [KO:K01367] [EC:3.4.22.52]
125024445  ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
125024463  [KO:K03347]
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
mcep00510  N-Glycan biosynthesis
mcep03050  Proteasome
mcep03060  Protein export
mcep04210  Apoptosis
KO pathway
ko04141   

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