KEGG   PATHWAY: zca04141
Entry
zca04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Zalophus californianus (California sea lion)
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
zca04141  Protein processing in endoplasmic reticulum
zca04141

Other DBs
GO: 0030433 0034976
Organism
Zalophus californianus (California sea lion) [GN:zca]
Gene
113908289  DERL2; derlin-2 isoform X1 [KO:K13989]
113908531  FBXO2; F-box only protein 2 [KO:K10099]
113908900  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
113909003  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
113909004  SEC23A; protein transport protein Sec23A isoform X1 [KO:K14006]
113909070  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
113909121  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
113909177  [KO:K13251]
113909364  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
113909409  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
113909972  LMAN1L; LOW QUALITY PROTEIN: protein ERGIC-53-like [KO:K10081]
113910395  SELENOS; selenoprotein S isoform X2 [KO:K14025]
113910509  ERO1A; ERO1-like protein alpha isoform X1 [KO:K10950] [EC:1.8.4.-]
113910992  CUL1; cullin-1 [KO:K03347]
113911152  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
113911459  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
113911560  [KO:K03868] [EC:2.3.2.32]
113911802  [KO:K08056] [EC:5.3.4.1]
113911876  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
113912489  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
113912694  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016]
113912735  LMAN1; protein ERGIC-53 [KO:K10080]
113912756  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
113913021  XBP1; X-box-binding protein 1 [KO:K09027]
113913187  DERL3; derlin-3 [KO:K13989]
113913284  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
113913621  [KO:K24349]
113913731  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
113913874  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
113914088  [KO:K14014]
113914145  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
113914166  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
113914238  [KO:K07342]
113914524  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
113914531  [KO:K04523]
113914802  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
113914864  TMEM258; LOW QUALITY PROTEIN: transmembrane protein 258 [KO:K26502]
113914883  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
113915008  CRYAB; alpha-crystallin B chain [KO:K09542]
113916195  [KO:K26502]
113916251  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
113916283  DNAJB1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
113916468  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
113916565  SEC62; translocation protein SEC62 [KO:K12275]
113916670  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
113916698  [KO:K26502]
113916784  CRYAA; LOW QUALITY PROTEIN: alpha-crystallin A chain [KO:K09541]
113916984  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
113916999  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
113917050  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
113917360  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
113917379  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
113917386  [KO:K03094]
113917431  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
113917437  [KO:K06689] [EC:2.3.2.23]
113917918  [KO:K09503]
113917935  [KO:K09502]
113918052  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
113918228  SSR3; translocon-associated protein subunit gamma [KO:K13251]
113918258  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
113918356  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
113918615  PRKCSH; glucosidase 2 subunit beta [KO:K08288]
113918790  CALR; calreticulin [KO:K08057]
113919288  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
113919289  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
113919460  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
113919904  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
113920049  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
113920119  HSPH1; heat shock protein 105 kDa [KO:K09485]
113920376  [KO:K26501]
113920409  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
113920631  UBXN4; UBX domain-containing protein 4 [KO:K24348]
113920779  [KO:K04079]
113920872  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
113921432  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
113921771  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
113921968  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X1 [KO:K03868] [EC:2.3.2.32]
113922031  HSP90B1; endoplasmin [KO:K09487]
113922091  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
113922229  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
113922246  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
113922476  OS9; protein OS-9 isoform X1 [KO:K10088]
113922522  [KO:K06689] [EC:2.3.2.23]
113922525  DNAJC1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
113922602  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
113922928  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
113923042  [KO:K09503]
113923427  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
113923430  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
113923590  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
113923808  SAR1A; GTP-binding protein SAR1a isoform X1 [KO:K07953] [EC:3.6.5.-]
113924053  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
113924428  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
113924468  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
113924587  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
113924616  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
113924851  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
113925156  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
113925297  WFS1; wolframin [KO:K14020]
113925312  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
113925386  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
113925388  [KO:K03094]
113925488  [KO:K03283]
113925734  [KO:K26501]
113925863  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
113925916  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
113926300  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
113926414  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA isoform X1 [KO:K01230] [EC:3.2.1.113]
113926534  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
113926587  TXNDC5; thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
113926588  SEC63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
113926602  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
113927015  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
113927073  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
113927090  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
113927191  [KO:K06689] [EC:2.3.2.23]
113927218  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
113927365  BAK1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
113927504  PRKN; LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
113927654  [KO:K04079]
113927727  HSPA1L; heat shock 70 kDa protein 1-like isoform X1 [KO:K03283]
113927729  HSPA1B; heat shock 70 kDa protein 1B [KO:K03283]
113928408  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
113928528  [KO:K03283]
113928769  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
113928894  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
113928932  [KO:K12668]
113928935  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
113928953  CANX; calnexin [KO:K08054]
113928992  LMAN2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
113929081  SAR1B; LOW QUALITY PROTEIN: GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
113929601  DERL1; derlin-1 isoform X1 [KO:K11519]
113929794  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
113929825  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
113930310  [KO:K24349]
113930568  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
113930576  [KO:K03283]
113930638  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
113930735  UBQLN2; ubiquilin-2 [KO:K04523]
113930887  [KO:K08057]
113930948  MAGT1; magnesium transporter protein 1 isoform X1 [KO:K19478]
113931467  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
113931888  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
113932153  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
113932175  UBQLN4; ubiquilin-4 [KO:K04523]
113932184  SSR2; translocon-associated protein subunit beta isoform X1 [KO:K13250]
113932281  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
113932475  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
113932654  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
113932795  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
113933604  KRTCAP2; keratinocyte-associated protein 2 isoform X1 [KO:K28202]
113934139  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
113934254  BAG1; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555]
113934257  [KO:K13525]
113934283  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
113934315  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
113934425  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
113934455  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
113934480  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
113934586  [KO:K23741]
113934587  [KO:K23741]
113934602  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
113934762  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
113935412  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
113935677  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
113935723  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
113935835  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
113935936  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
113936268  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
113936434  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
113936991  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
113936998  SEL1L2; protein sel-1 homolog 2 isoform X1 [KO:K14026]
113936999  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
113937205  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
113937285  EDEM2; LOW QUALITY PROTEIN: ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
113937296  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
113937329  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
113937393  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
113937446  [KO:K03094]
113937483  SEC23B; protein transport protein Sec23B [KO:K14006]
113937508  MOGS; LOW QUALITY PROTEIN: mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
113937517  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
113937908  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
113937978  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
113938072  [KO:K03094]
113938319  [KO:K04079]
113938525  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
113938760  FBXO6; F-box only protein 6 isoform X1 [KO:K10100]
113939180  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
113939240  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
113939265  [KO:K10839]
113939396  UBE2G1; ubiquitin-conjugating enzyme E2 G1 isoform X1 [KO:K10575] [EC:2.3.2.23]
113939825  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
118355935  [KO:K04741] [EC:3.4.22.-]
118356458  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
118356791  [KO:K09502]
118356931  [KO:K09502]
118357042  [KO:K09502]
118357183  [KO:K03283]
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
zca00510  N-Glycan biosynthesis
zca03050  Proteasome
zca03060  Protein export
zca04210  Apoptosis
zca04930  Type II diabetes mellitus
zca05010  Alzheimer disease
zca05012  Parkinson disease
zca05020  Prion disease
KO pathway
ko04141   

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