KEGG   PATHWAY: pyu04141
Entry
pyu04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Puma yagouaroundi (jaguarundi)
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
pyu04141  Protein processing in endoplasmic reticulum
pyu04141

Other DBs
GO: 0030433 0034976
Organism
Puma yagouaroundi (jaguarundi) [GN:pyu]
Gene
121009861  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
121010330  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
121010516  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
121010523  CANX; calnexin [KO:K08054]
121010703  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
121010712  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
121010713  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
121010781  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
121010793  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
121011632  LMAN2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082]
121011839  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
121011871  [KO:K03283]
121012067  [KO:K09502]
121012228  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
121012270  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
121012379  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
121012505  SEL1L2; protein sel-1 homolog 2 isoform X1 [KO:K14026]
121012517  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
121012538  SEC23B; protein transport protein Sec23B [KO:K14006]
121012646  HSPH1; heat shock protein 105 kDa [KO:K09485]
121013069  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
121013412  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
121013593  [KO:K09502]
121013900  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
121014014  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
121014055  CALR; calreticulin isoform X1 [KO:K08057]
121014057  RAD23A; UV excision repair protein RAD23 homolog A [KO:K10839]
121014102  DNAJB1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
121014229  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
121014730  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
121014815  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
121015188  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
121015275  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
121015462  UBQLN4; LOW QUALITY PROTEIN: ubiquilin-4 [KO:K04523]
121015463  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
121015619  SSR2; translocon-associated protein subunit beta [KO:K13250]
121016075  DERL2; derlin-2 isoform X1 [KO:K13989]
121016098  UBE2G1; ubiquitin-conjugating enzyme E2 G1 isoform X1 [KO:K10575] [EC:2.3.2.23]
121016294  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
121017014  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
121018057  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
121018067  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
121018188  [KO:K09502]
121018751  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
121018934  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
121019225  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
121019768  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
121020066  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
121020233  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
121020261  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
121020512  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
121020525  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
121020679  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
121020796  [KO:K04741] [EC:3.4.22.-]
121020843  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
121021089  FBXO2; F-box only protein 2 isoform X1 [KO:K10099]
121021130  FBXO6; F-box only protein 6 [KO:K10100]
121021147  UBQLN3; ubiquilin-3 [KO:K04523]
121021519  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
121022289  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
121022290  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
121022323  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
121022360  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
121022385  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
121022388  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
121022649  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
121022672  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
121023041  DERL3; derlin-3 isoform X1 [KO:K13989]
121023150  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
121023201  [KO:K09502]
121023322  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
121023433  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
121023455  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
121023671  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
121023712  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:2.3.2.27]
121023931  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
121023984  UBXN1; UBX domain-containing protein 1 [KO:K24348]
121024611  UBQLN2; ubiquilin-2 [KO:K04523]
121024619  [KO:K23741]
121025044  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
121025082  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
121025238  [KO:K03283]
121025365  MAGT1; magnesium transporter protein 1 isoform X1 [KO:K19478]
121026120  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
121026121  YY2; transcription factor YY2 [KO:K07765] [EC:3.4.24.85]
121026457  [KO:K03283]
121026473  [KO:K03283]
121027783  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
121028148  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
121028153  CUL1; cullin-1 isoform X1 [KO:K03347]
121029294  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
121029518  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
121029617  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
121029775  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
121029862  SEC63; translocation protein SEC63 homolog [KO:K09540]
121029881  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
121029985  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
121030385  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
121030494  UBXN4; UBX domain-containing protein 4 [KO:K24348]
121030539  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
121030580  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
121030749  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
121030786  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
121030939  [KO:K03283]
121031106  DNAJB2; dnaJ homolog subfamily B member 2 [KO:K09508]
121031155  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
121031416  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
121031477  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 isoform X1 [KO:K12668]
121031681  SEC23A; protein transport protein Sec23A isoform X1 [KO:K14006]
121031767  ERO1A; ERO1-like protein alpha isoform X1 [KO:K10950] [EC:1.8.4.-]
121031821  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
121031936  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
121032143  SEL1L; protein sel-1 homolog 1 [KO:K14026]
121032208  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
121032306  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
121032687  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
121032865  LMAN1; protein ERGIC-53 isoform X1 [KO:K10080]
121033019  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
121033436  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
121033557  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
121033578  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
121033590  [KO:K07342]
121033886  SSR3; translocon-associated protein subunit gamma [KO:K13251]
121033976  SEC62; translocation protein SEC62 [KO:K12275]
121034116  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
121034536  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
121034622  [KO:K13525]
121034737  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
121034792  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
121034988  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
121035178  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
121035385  CRYAB; alpha-crystallin B chain [KO:K09542]
121035496  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
121035557  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
121035596  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
121035859  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
121035892  CRYAA; alpha-crystallin A chain [KO:K09541]
121036101  [KO:K03283]
121036653  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
121036736  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
121036770  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
121036796  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
121036923  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
121037112  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
121037657  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
121037787  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
121038137  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
121038181  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
121038665  WFS1; wolframin [KO:K14020]
121039743  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
121039794  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
121040293  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
121040318  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
121040478  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
121040515  HSP90B1; endoplasmin [KO:K09487]
121040866  OS9; protein OS-9 isoform X1 [KO:K10088]
121040880  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
121041264  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
121041322  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
121041608  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
121041996  LMAN1L; protein ERGIC-53-like [KO:K10081]
121042499  SELENOS; selenoprotein S [KO:K14025]
121042689  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
121042927  DERL1; derlin-1 isoform X1 [KO:K11519]
121043316  TMEM258; transmembrane protein 258 [KO:K26502]
121043644  [KO:K14014]
121044096  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
121044187  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
121044242  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
121044262  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
121044477  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
121045242  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
121045328  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
121045340  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
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
pyu00510  N-Glycan biosynthesis
pyu03050  Proteasome
pyu03060  Protein export
pyu04210  Apoptosis
pyu04930  Type II diabetes mellitus
pyu05010  Alzheimer disease
pyu05012  Parkinson disease
pyu05020  Prion disease
KO pathway
ko04141   

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