KEGG   PATHWAY: umr04141
Entry
umr04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Ursus maritimus (polar bear)
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
umr04141  Protein processing in endoplasmic reticulum
umr04141

Other DBs
GO: 0030433 0034976
Organism
Ursus maritimus (polar bear) [GN:umr]
Gene
103676291  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
103669615  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
103678507  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
103674548  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
103676162  SEC62; translocation protein SEC62 [KO:K12275]
103675841  SEC63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
103669619  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
103669902  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
103681026  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
103666588  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
103660628  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
103680114  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
103675818  MAGT1; magnesium transporter protein 1 [KO:K19478]
103681370  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
103661732  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
103678170  TMEM258; transmembrane protein 258 [KO:K26502]
103656903  transmembrane protein 258-like [KO:K26502]
103671412  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
103676561  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
103680517  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
103663776  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
103662319  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
103670323  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
103676056  DNAJB11; dnaJ homolog subfamily B member 11 isoform X1 [KO:K09517]
103664228  DNAJC1; dnaJ homolog subfamily C member 1 isoform X1 [KO:K09521]
103657649  DNAJC3; dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
103659645  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
103676491  HSP90B1; endoplasmin [KO:K09487]
103678188  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
103678705  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
103679986  CANX; calnexin [KO:K08054]
103673527  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
103682146  CALR; calreticulin [KO:K08057]
103663450  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA isoform X1 [KO:K01230] [EC:3.2.1.113]
103666782  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
103680866  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
103675308  LOW QUALITY PROTEIN: endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
103664446  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
103676013  LMAN1; protein ERGIC-53 [KO:K10080]
103678990  LMAN1L; protein ERGIC-53-like [KO:K10081]
103671655  PREB; prolactin regulatory element-binding protein [KO:K14003]
103663811  SAR1B; GTP-binding protein SAR1b isoform X1 [KO:K07953] [EC:3.6.5.-]
103667790  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
103669559  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
103677144  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
103665361  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
103665869  SEC23A; protein transport protein Sec23A [KO:K14006]
103680529  SEC23B; protein transport protein Sec23B [KO:K14006]
103661736  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
103661776  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
103663810  SEC24A; protein transport protein Sec24A [KO:K14007]
103667832  SEC24C; protein transport protein Sec24C [KO:K14007]
103657647  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
103658560  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
103669652  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
103669810  ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
103668803  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
103666000  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
103669067  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
103660384  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
103661192  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
103664933  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
103672910  ERO1A; ERO1-like protein alpha isoform X2 [KO:K10950] [EC:1.8.4.-]
103662007  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
103656940  OS9; protein OS-9 isoform X1 [KO:K10088]
103671255  ERLEC1; endoplasmic reticulum lectin 1 isoform X2 [KO:K14008]
103664937  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
103668362  SSR2; translocon-associated protein subunit beta [KO:K13250]
103677571  SSR3; translocon-associated protein subunit gamma [KO:K13251]
103679697  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
103679671  BCAP31; B-cell receptor-associated protein 31 isoform X1 [KO:K14009]
103681245  TRAM1; translocating chain-associated membrane protein 1 isoform X1 [KO:K14010]
103661769  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
103661606  DERL1; derlin-1 isoform X1 [KO:K11519]
103660433  DERL2; derlin-2 isoform X1 [KO:K13989]
103669280  DERL3; derlin-3 isoform X2 [KO:K13989]
103667674  UBXN4; UBX domain-containing protein 4 [KO:K24348]
103678194  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
103671933  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
103681686  UBXN6; UBX domain-containing protein 6 [KO:K14011]
103659080  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
103674499  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
121104751  small VCP/p97-interacting protein [KO:K14014]
103667114  transitional endoplasmic reticulum ATPase [KO:K13525]
103665994  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
103669252  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
103662283  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
103658293  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
103682293  LOW QUALITY PROTEIN: heat shock 70 kDa protein 1 [KO:K03283]
103682294  LOW QUALITY PROTEIN: heat shock 70 kDa protein 1-like [KO:K03283]
103682295  heat shock 70 kDa protein 1-like [KO:K03283]
121106055  heat shock 70 kDa protein 1 isoform X1 [KO:K03283]
103667028  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
103666927  LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 1-like [KO:K09502]
103673814  DNAJA2; dnaJ homolog subfamily A member 2 isoform X1 [KO:K09503]
103682112  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
103662743  DNAJB2; dnaJ homolog subfamily B member 2 isoform X2 [KO:K09508]
103667810  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
103660531  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
103677478  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
103671648  DNAJC5G; LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 5G [KO:K09525]
103664050  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
103656761  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
103656990  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
103664344  HSPH1; LOW QUALITY PROTEIN: heat shock protein 105 kDa [KO:K09485]
103667107  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
103663976  BAG2; BAG family molecular chaperone regulator 2 isoform X1 [KO:K09556]
103680264  HSPBP1; hsp70-binding protein 1 isoform X2 [KO:K09562]
103680761  CRYAA; alpha-crystallin A chain [KO:K09541]
103662396  CRYAB; alpha-crystallin B chain [KO:K09542]
103657752  YOD1; ubiquitin thioesterase OTU1 isoform X2 [KO:K13719] [EC:3.1.2.-]
103666999  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
103682145  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
103678550  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
103680054  UBQLN3; ubiquilin-3 [KO:K04523]
103674305  UBQLN2; ubiquilin-2 [KO:K04523]
103667645  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
103668691  ubiquilin-4 [KO:K04523]
103679804  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
103670119  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
103666667  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
103671855  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
103671936  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
103659605  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X2 [KO:K05638]
103676372  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
103657105  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
103656598  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
103676164  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
103658290  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
103682282  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
103658294  WFS1; LOW QUALITY PROTEIN: wolframin [KO:K14020]
103662102  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
103668263  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
103659239  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
103665903  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
103675245  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
103663496  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X2 [KO:K04426] [EC:2.7.11.25]
103681888  MAP2K7; LOW QUALITY PROTEIN: dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
103676947  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
103679990  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
103667893  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
103657107  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
103665481  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
103679450  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
103669131  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
103662441  LOW QUALITY PROTEIN: caspase-12 [KO:K04741] [EC:3.4.22.-]
103662241  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
103659952  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
103682055  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
103660418  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
103680695  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
103679571  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
103682276  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X2 [KO:K10666] [EC:2.3.2.27]
103681087  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
103659981  SELENOS; selenoprotein S [KO:K14025]
103670573  SEL1L; protein sel-1 homolog 1 [KO:K14026]
103674547  SEL1L2; protein sel-1 homolog 2 isoform X3 [KO:K14026]
103673772  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
103673897  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
103668458  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
103663765  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
103677950  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X2 [KO:K06689] [EC:2.3.2.23]
103667731  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
103660821  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
103674160  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X1 [KO:K03868] [EC:2.3.2.32]
103668956  CUL1; cullin-1 [KO:K03347]
103663817  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
103666862  FBXO2; F-box only protein 2 [KO:K10099]
103666655  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
umr00510  N-Glycan biosynthesis
umr03050  Proteasome
umr03060  Protein export
umr04210  Apoptosis
umr04930  Type II diabetes mellitus
umr05010  Alzheimer disease
umr05012  Parkinson disease
umr05020  Prion disease
KO pathway
ko04141   
LinkDB

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