KEGG   PATHWAY: ssc04141
Entry
ssc04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Sus scrofa (pig)
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
ssc04141  Protein processing in endoplasmic reticulum
ssc04141

Other DBs
GO: 0030433 0034976
Organism
Sus scrofa (pig) [GN:ssc]
Gene
100155306  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
100511831  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
100511362  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
100517415  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
100125961  SEC62; translocation protein SEC62 [KO:K12275]
100152304  SEC63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
397606  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 precursor [KO:K12666]
100154432  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 precursor [KO:K12667]
396984  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
397385  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit precursor [KO:K12670]
100511525  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
100526009  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
100517568  MAGT1; magnesium transporter protein 1 [KO:K19478]
106504082  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
100517665  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
100517573  TMEM258; transmembrane protein 258 [KO:K26502]
100513204  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
100523493  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
100154945  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
100626868  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
100516870  HYOU1; hypoxia up-regulated protein 1 isoform X2 [KO:K09486]
407060  HSPA5; 78 kDa glucose-regulated protein isoform X2 [KO:K09490] [EC:3.6.4.10]
100152894  DNAJB11; dnaJ homolog subfamily B member 11 precursor [KO:K09517]
100519794  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
100154166  DNAJC3; dnaJ homolog subfamily C member 3 precursor [KO:K09523]
100523288  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
397191  HSP90B1; endoplasmin precursor [KO:K09487]
396938  GANAB; neutral alpha-glucosidase AB precursor [KO:K05546] [EC:3.2.1.207]
100516540  PRKCSH; glucosidase 2 subunit beta precursor [KO:K08288]
100519184  CANX; calnexin precursor [KO:K08054]
100156204  PDIA3; protein disulfide-isomerase A3 precursor [KO:K08056] [EC:5.3.4.1]
100381266  CALR; calreticulin precursor [KO:K08057]
396919  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
100154147  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
100524150  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
100627465  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
397594  LMAN2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
100153007  LMAN1; protein ERGIC-53 [KO:K10080]
100158128  LMAN1L; protein ERGIC-53-like isoform X2 [KO:K10081]
100522734  PREB; prolactin regulatory element-binding protein [KO:K14003]
595115  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
494017  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
100739864  SEC13; protein SEC13 homolog [KO:K14004]
100511210  SEC31A; protein transport protein Sec31A isoform X14 [KO:K14005]
100513277  SEC31B; protein transport protein Sec31B isoform X7 [KO:K14005]
100155766  SEC23A; protein transport protein Sec23A [KO:K14006]
100524115  SEC23B; protein transport protein Sec23B [KO:K14006]
100152996  SEC24C; protein transport protein Sec24C isoform X2 [KO:K14007]
100511986  SEC24A; protein transport protein Sec24A [KO:K14007]
100622653  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
100739491  SEC24D; protein transport protein Sec24D [KO:K14007]
100155117  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
100154594  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
100738285  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
100621927  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
100625293  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
110255932  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
100522203  PDIA4; protein disulfide-isomerase A4 precursor [KO:K09582] [EC:5.3.4.1]
100499564  PDIA6; protein disulfide-isomerase A6 precursor [KO:K09584] [EC:5.3.4.1]
100156625  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
100156354  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
100192435  ERO1A; ERO1-like protein alpha precursor [KO:K10950] [EC:1.8.4.-]
100154974  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
110260688  OS9; LOW QUALITY PROTEIN: protein OS-9 [KO:K10088]
100517335  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
100152689  SSR1; signal sequence receptor, alpha [KO:K13249]
100154318  SSR2; translocon-associated protein subunit beta [KO:K13250]
100625983  SSR3; translocon-associated protein subunit gamma [KO:K13251]
100523300  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
100520774  BCAP31; B-cell receptor-associated protein 31 isoform X2 [KO:K14009]
100151996  TRAM1; translocating chain-associated membrane protein 1 isoform X1 [KO:K14010]
100511093  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
100626802  DERL1; derlin-1 [KO:K11519]
100155947  DERL3; derlin-3 isoform X1 [KO:K13989]
110256099  DERL2; derlin-2 [KO:K13989]
100737637  UBXN4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
100621165  UBXN1; UBX domain-containing protein 1 [KO:K24348]
100522609  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
100524481  UBXN6; UBX domain-containing protein 6 isoform X2 [KO:K14011]
100513050  UBXN8; UBX domain-containing protein 8 [KO:K24351]
100513186  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
100625679  SVIP; small VCP/p97-interacting protein [KO:K14014]
397524  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
100625565  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
100153062  UFD1; ubiquitin fusion degradation protein 1 homolog isoform X2 [KO:K14016]
396648  HSP70.2; heat shock 70 kDa protein 1B [KO:K03283]
100144518  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
396906  HSPA6; heat shock 70 kDa protein [KO:K03283]
100511890  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
100512008  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
100521133  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
396741  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
100522168  DNAJB2; dnaJ homolog subfamily B member 2 isoform X6 [KO:K09508]
100156234  DNAJB12; dnaJ homolog subfamily B member 12 isoform X2 [KO:K09518]
100157247  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
100621635  DNAJC5; dnaJ homolog subfamily C member 5 isoform X1 [KO:K09525]
110260046  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
397028  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
396742  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
100048931  HSPH1; heat shock protein 105 kDa [KO:K09485]
100517839  HSPA4L; LOW QUALITY PROTEIN: heat shock 70 kDa protein 4L [KO:K09485]
100513225  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
100522554  BAG2; BAG family molecular chaperone regulator 2 isoform X1 [KO:K09556]
100518376  HSPBP1; hsp70-binding protein 1 [KO:K09562]
110256286  CRYAA; alpha-crystallin A chain [KO:K09541]
100519789  CRYAB; alpha-crystallin B chain [KO:K09542]
100520400  YOD1; ubiquitin thioesterase OTU1 isoform X2 [KO:K13719] [EC:3.1.2.-]
100511733  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
100153668  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
100524483  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
100519020  UBQLN4; ubiquilin-4 [KO:K04523]
100622592  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
100525764  UBQLN3; ubiquilin-3 [KO:K04523]
110257666  UBQLN2; ubiquilin-2 [KO:K04523]
100522951  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
100144422  ATXN3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
100737278  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
100513348  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
100155472  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
100516343  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X4 [KO:K05638]
100144302  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
100522679  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
100240743  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
100049703  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
100158076  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
100144516  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
100621070  WFS1; LOW QUALITY PROTEIN: wolframin [KO:K14020]
100516674  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
102165056  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
100217386  XBP1; X-box-binding protein 1 isoform XBP1(U) [KO:K09027]
100510978  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
100513840  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
100622769  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
100520418  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X5 [KO:K04431] [EC:2.7.12.2]
396610  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
100521736  MAPK10; mitogen-activated protein kinase 10 isoform X3 [KO:K04440] [EC:2.7.11.24]
396609  MAPK9; mitogen-activated protein kinase 9 isoform X4 [KO:K04440] [EC:2.7.11.24]
396633  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
397543  BAK1; bcl-2 homologous antagonist/killer isoform X2 [KO:K14021]
397027  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
397393  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
100170772  MARCH6; E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
768104  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
100523610  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
100522828  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
100624139  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
100514334  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X2 [KO:K10601] [EC:2.3.2.27]
100144528  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
100157569  RNF185; E3 ubiquitin-protein ligase RNF185 isoform X2 [KO:K10666] [EC:2.3.2.27]
100151836  SELENOS; selenoprotein S [KO:K14025]
100520751  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
100152967  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
100622579  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
100516197  AMFR; LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
100511251  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
100524597  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
396580  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
780418  UBE2D3; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
780419  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
733673  PRKN; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
110260638  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X1 [KO:K03868] [EC:2.3.2.32]
100524037  CUL1; cullin-1 [KO:K03347]
100520183  LOW QUALITY PROTEIN: S-phase kinase-associated protein 1 [KO:K03094]
110259483  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
100511640  FBXO2; F-box only protein 2 [KO:K10099]
100524148  FBXO6; 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
ssc00510  N-Glycan biosynthesis
ssc03050  Proteasome
ssc03060  Protein export
ssc04210  Apoptosis
ssc04930  Type II diabetes mellitus
ssc05010  Alzheimer disease
ssc05012  Parkinson disease
ssc05020  Prion disease
KO pathway
ko04141   
LinkDB

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