KEGG   PATHWAY: mmur04141
Entry
mmur04141                   Pathway                                
Name
Protein processing in endoplasmic reticulum - Microcebus murinus (gray mouse lemur)
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
mmur04141  Protein processing in endoplasmic reticulum
mmur04141

Other DBs
GO: 0030433 0034976
Organism
Microcebus murinus (gray mouse lemur) [GN:mmur]
Gene
105854819  LMAN1L; LOW QUALITY PROTEIN: protein ERGIC-53-like [KO:K10081]
105854849  [KO:K03283]
105854916  SVIP; small VCP/p97-interacting protein [KO:K14014]
105855261  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X2 [KO:K10086]
105855702  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
105855915  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
105856287  STUB1; E3 ubiquitin-protein ligase CHIP isoform X1 [KO:K09561] [EC:2.3.2.27]
105856373  [KO:K10080]
105856492  ERO1B; ERO1-like protein beta isoform X1 [KO:K10976] [EC:1.8.4.-]
105856630  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
105857017  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
105857227  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
105857621  UBXN4; UBX domain-containing protein 4 [KO:K24348]
105857667  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
105858049  [KO:K14004]
105858143  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
105858153  RNF5; LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
105858167  [KO:K03283]
105858168  [KO:K03283]
105858417  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
105858535  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
105858549  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
105859496  DERL1; derlin-1 isoform X1 [KO:K11519]
105859524  HSP90AB1; LOW QUALITY PROTEIN: heat shock protein HSP 90-beta [KO:K04079]
105859677  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
105860020  [KO:K09502]
105860097  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
105860631  LMAN1; protein ERGIC-53 [KO:K10080]
105861065  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
105861175  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
105861442  BAK1; bcl-2 homologous antagonist/killer isoform X1 [KO:K14021]
105861700  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
105861829  [KO:K10839]
105861836  WFS1; wolframin isoform X1 [KO:K14020]
105862473  [KO:K04741] [EC:3.4.22.-]
105862491  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
105862513  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
105862942  CRYAB; alpha-crystallin B chain [KO:K09542]
105863173  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
105863415  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
105863616  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
105863767  [KO:K03853] [EC:3.4.22.53]
105863853  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
105864018  [KO:K13984] [EC:5.3.4.1]
105864291  [KO:K04440] [EC:2.7.11.24]
105864738  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
105864826  UBQLN2; ubiquilin-2 [KO:K04523]
105864878  [KO:K10956]
105864962  SEC63; translocation protein SEC63 homolog [KO:K09540]
105865178  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
105865189  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
105865590  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
105865602  BAX; apoptosis regulator BAX isoform X2 [KO:K02159]
105865754  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
105865932  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
105866009  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
105866031  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
105866157  MAGT1; magnesium transporter protein 1 isoform X1 [KO:K19478]
105866461  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
105866540  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
105867307  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
105867310  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
105867670  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
105867912  SEL1L; protein sel-1 homolog 1 [KO:K14026]
105867917  DNAJB1; dnaJ homolog subfamily B member 1 isoform X1 [KO:K09507]
105868536  RRBP1; ribosome-binding protein 1 isoform X2 [KO:K14000]
105868875  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
105869064  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
105869133  YOD1; ubiquitin thioesterase OTU1 isoform X1 [KO:K13719] [EC:3.1.2.-]
105869403  EDEM1; LOW QUALITY PROTEIN: ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
105869842  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
105869977  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
105869985  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
105869986  SAR1B; LOW QUALITY PROTEIN: GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
105870165  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
105870200  SAR1A; GTP-binding protein SAR1a isoform X1 [KO:K07953] [EC:3.6.5.-]
105870282  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
105870895  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
105870952  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
105871187  PLAA; LOW QUALITY PROTEIN: phospholipase A-2-activating protein [KO:K14018]
105871287  SELENOS; selenoprotein S isoform X1 [KO:K14025]
105871989  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
105872323  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
105872339  SEC24B; LOW QUALITY PROTEIN: protein transport protein Sec24B [KO:K14007]
105872529  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
105872602  SEC31A; LOW QUALITY PROTEIN: protein transport protein Sec31A [KO:K14005]
105872778  SEC23A; LOW QUALITY PROTEIN: protein transport protein Sec23A [KO:K14006]
105872915  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
105873143  UBQLN4; ubiquilin-4 [KO:K04523]
105873149  SSR2; translocon-associated protein subunit beta isoform X1 [KO:K13250]
105873177  KRTCAP2; keratinocyte-associated protein 2 isoform X1 [KO:K28202]
105873323  CUL1; cullin-1 [KO:K03347]
105873328  PDIA4; protein disulfide-isomerase A4 isoform X1 [KO:K09582] [EC:5.3.4.1]
105873559  HSPA6; heat shock 70 kDa protein 6 [KO:K03283]
105873568  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
105873598  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
105873780  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
105873872  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
105873892  [KO:K06689] [EC:2.3.2.23]
105873925  UBXN6; UBX domain-containing protein 6 [KO:K14011]
105874152  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718] [EC:2.4.1.-]
105874172  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
105874327  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
105874385  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
105874693  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
105874790  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
105874813  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
105875192  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
105875581  HSP90B1; endoplasmin [KO:K09487]
105875610  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
105875763  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
105876026  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
105876213  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
105876271  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
105876638  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
105876665  [KO:K12666]
105876728  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
105877199  HSPBP1; hsp70-binding protein 1 isoform X1 [KO:K09562]
105877220  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
105877546  [KO:K08057]
105877639  UBE2J2; ubiquitin-conjugating enzyme E2 J2 isoform X1 [KO:K04554] [EC:2.3.2.23]
105877739  UFD1; ubiquitin fusion degradation protein 1 homolog [KO:K14016]
105877818  DERL3; derlin-3 isoform X1 [KO:K13989]
105877984  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
105877994  OS9; protein OS-9 isoform X3 [KO:K10088]
105878412  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
105878510  SYVN1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
105878513  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
105878588  UBXN1; UBX domain-containing protein 1 isoform X1 [KO:K24348]
105878594  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
105878625  TMEM258; transmembrane protein 258 [KO:K26502]
105878682  ERP29; endoplasmic reticulum resident protein 29 isoform X1 [KO:K09586]
105879030  PDIA6; LOW QUALITY PROTEIN: protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
105879115  CRYAA; alpha-crystallin A chain isoform X1 [KO:K09541]
105879130  [KO:K09517]
105879155  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
105879186  SEC62; translocation protein SEC62 [KO:K12275]
105879245  DERL2; derlin-2 isoform X1 [KO:K13989]
105879493  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
105879515  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
105879629  SEC23B; LOW QUALITY PROTEIN: protein transport protein Sec23B [KO:K14006]
105879653  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
105880075  SEL1L2; protein sel-1 homolog 2 isoform X1 [KO:K14026]
105880242  EIF2S1; LOW QUALITY PROTEIN: eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
105881053  [KO:K06689] [EC:2.3.2.23]
105881610  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
105881817  [KO:K14006]
105882049  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
105882249  MAN1B1; LOW QUALITY PROTEIN: endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
105882282  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
105882360  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
105882721  FBXO6; F-box only protein 6 [KO:K10100]
105882723  FBXO2; F-box only protein 2 [KO:K10099]
105882985  [KO:K12666]
105882997  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
105882998  [KO:K09584] [EC:5.3.4.1]
105883030  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
105883217  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
105883324  SEC24D; protein transport protein Sec24D [KO:K14007]
105883483  UBQLN3; ubiquilin-3 [KO:K04523]
105883643  SSR3; translocon-associated protein subunit gamma [KO:K13251]
105883823  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
105883988  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
105884330  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
105884393  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
105884404  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525]
105884584  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
105884953  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
105884966  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
105885064  DNAJB11; dnaJ homolog subfamily B member 11 isoform X1 [KO:K09517]
105885170  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
105885203  CANX; calnexin isoform X1 [KO:K08054]
105885495  CALR; calreticulin [KO:K08057]
105885497  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
105885560  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
105885655  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
105885821  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
105885853  MARCH6; E3 ubiquitin-protein ligase MARCH6 isoform X2 [KO:K10661] [EC:2.3.2.27]
105885928  [KO:K10956]
105886029  UBE4B; LOW QUALITY PROTEIN: ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
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
mmur00510  N-Glycan biosynthesis
mmur03050  Proteasome
mmur03060  Protein export
mmur04210  Apoptosis
mmur04930  Type II diabetes mellitus
mmur05010  Alzheimer disease
mmur05012  Parkinson disease
mmur05020  Prion disease
KO pathway
ko04141   

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