KEGG   PATHWAY: pcoo04141
Entry
pcoo04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Puma concolor (puma)
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
pcoo04141  Protein processing in endoplasmic reticulum
pcoo04141

Other DBs
GO: 0030433 0034976
Organism
Puma concolor (puma) [GN:pcoo]
Gene
112867989  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
112858032  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
112848820  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
112860078  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
112849303  SEC62; translocation protein SEC62 [KO:K12275]
112862110  SEC63; translocation protein SEC63 homolog [KO:K09540]
112857530  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
112869513  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
112855312  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
112868210  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
112850051  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
112866106  STT3A; LOW QUALITY PROTEIN: dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
112870819  MAGT1; magnesium transporter protein 1 [KO:K19478]
112869963  TUSC3; tumor suppressor candidate 3 [KO:K19478]
112858151  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
112852145  TMEM258; transmembrane protein 258 [KO:K26502]
112869344  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
112863331  CKAP4; LOW QUALITY PROTEIN: cytoskeleton-associated protein 4 [KO:K13999]
112869594  RRBP1; ribosome-binding protein 1 [KO:K14000]
112861112  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
112865838  HYOU1; hypoxia up-regulated protein 1 [KO:K09486]
112850502  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
112849511  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
112867970  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
112860265  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
112871764  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
112863931  HSP90B1; endoplasmin [KO:K09487]
112852204  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
112861376  CANX; calnexin isoform X1 [KO:K08054]
112854989  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
112852509  CALR; calreticulin [KO:K08057]
112862660  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
112871318  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
112871851  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
112860956  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
112861148  LMAN2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
112868252  LMAN1; protein ERGIC-53 [KO:K10080]
112855515  LMAN1L; LOW QUALITY PROTEIN: protein ERGIC-53-like [KO:K10081]
112864206  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
112860365  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
112854592  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
112858369  SEC13; LOW QUALITY PROTEIN: protein SEC13 homolog [KO:K14004]
112858585  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
112854448  SEC31B; protein transport protein Sec31B [KO:K14005]
112855358  SEC23A; protein transport protein Sec23A [KO:K14006]
112869703  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
112858349  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
112858797  SEC24B; protein transport protein Sec24B [KO:K14007]
112854486  SEC24C; protein transport protein Sec24C [KO:K14007]
112860912  SEC24A; protein transport protein Sec24A [KO:K14007]
112871877  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
112860755  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
112858766  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
112868785  ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
112857746  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
112864625  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
112866487  PDIA4; LOW QUALITY PROTEIN: protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
112864037  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
112870163  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
112853662  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
112855769  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
112851521  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
112863629  OS9; protein OS-9 isoform X1 [KO:K10088]
112868801  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
112853758  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
112849311  SSR3; translocon-associated protein subunit gamma [KO:K13251]
112870602  SSR4; translocon-associated protein subunit delta [KO:K04571]
112870597  BCAP31; B-cell receptor-associated protein 31 isoform X1 [KO:K14009]
112858616  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
112859504  TRAM1; translocating chain-associated membrane protein 1 isoform X1 [KO:K14010]
112859784  DERL1; derlin-1 isoform X1 [KO:K11519]
112857083  DERL2; derlin-2 isoform X1 [KO:K13989]
112870521  DERL3; derlin-3 isoform X1 [KO:K13989]
112852001  UBXN1; UBX domain-containing protein 1 [KO:K24348]
112848538  UBXN4; UBX domain-containing protein 4 [KO:K24348]
112864054  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
112868426  UBXN6; UBX domain-containing protein 6 [KO:K14011]
112869905  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
112869563  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
112864929  SVIP; small VCP/p97-interacting protein [KO:K14014]
112871974  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
112864622  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
112870150  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
112865282  HSPA8; LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein [KO:K03283]
112853784  heat shock 70 kDa protein 1-like [KO:K03283]
112853785  heat shock 70 kDa protein 1-like [KO:K03283]
112848374  LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 1-like [KO:K09502]
112848630  DNAJA1; LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 1 [KO:K09502]
112857225  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
112852316  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
112867160  DNAJB2; dnaJ homolog subfamily B member 2 [KO:K09508]
112854647  DNAJB12; dnaJ homolog subfamily B member 12 [KO:K09518]
112864107  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
112859618  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
112855434  HSP90AA1; heat shock protein HSP 90-alpha isoform X1 [KO:K04079]
112862791  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
112858020  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
112861353  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
112855122  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
112862095  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
112851116  HSPBP1; hsp70-binding protein 1 [KO:K09562]
112864906  CRYAB; alpha-crystallin B chain [KO:K09542]
112857647  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
112865068  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
112852558  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
112848425  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
112868721  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
112851696  UBQLN4; ubiquilin-4 [KO:K04523]
112871069  UBQLN2; ubiquilin-2 isoform X1 [KO:K04523]
112865558  UBQLN3; ubiquilin-3 [KO:K04523]
112849534  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
112855861  ATXN3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
112848937  UBE4B; ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
112864237  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
112855314  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
112868459  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
112863930  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
112851102  PPP1R15A; LOW QUALITY PROTEIN: protein phosphatase 1 regulatory subunit 15A [KO:K14019]
112863246  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
112868295  BCL2; LOW QUALITY PROTEIN: apoptosis regulator Bcl-2 [KO:K02161]
112864801  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
112853637  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
112858265  WFS1; wolframin [KO:K14020]
112857556  MBTPS1; LOW QUALITY PROTEIN: membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
112871651  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
112870154  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
112864460  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
112860577  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
112862885  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
112859092  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
112854846  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
112861322  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
112861960  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
112851928  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
112865165  caspase-12-like [KO:K04741] [EC:3.4.22.-]
112860247  MARCH6; E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
112862209  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
112871961  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
112856954  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
112849491  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
112852018  SYVN1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
112853537  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
112870337  RNF185; E3 ubiquitin-protein ligase RNF185 isoform X1 [KO:K10666] [EC:2.3.2.27]
112855334  SELENOS; selenoprotein S [KO:K14025]
112855311  SEL1L; protein sel-1 homolog 1 [KO:K14026]
112869244  SEL1L2; protein sel-1 homolog 2 isoform X1 [KO:K14026]
112857576  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
112857373  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
112858368  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
112854458  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
112860925  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
112862211  PRKN; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
112863270  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
112866485  CUL1; cullin-1 [KO:K03347]
112861435  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
112861537  FBXO2; F-box only protein 2 [KO:K10099]
112861547  LOW QUALITY PROTEIN: F-box only protein 6-like [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
pcoo00510  N-Glycan biosynthesis
pcoo03050  Proteasome
pcoo03060  Protein export
pcoo04210  Apoptosis
pcoo04930  Type II diabetes mellitus
pcoo05010  Alzheimer disease
pcoo05012  Parkinson disease
pcoo05020  Prion disease
KO pathway
ko04141   
LinkDB

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