KEGG   PATHWAY: mmf04141
Entry
mmf04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Molossus molossus (Pallas's mastiff bat)
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
mmf04141  Protein processing in endoplasmic reticulum
mmf04141

Other DBs
GO: 0030433 0034976
Organism
Molossus molossus (Pallas's mastiff bat) [GN:mmf]
Gene
118618133  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
118615242  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
118629784  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
118620017  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
118617077  SEC62; translocation protein SEC62 isoform X1 [KO:K12275]
118621276  SEC63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
118618509  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 isoform X1 [KO:K12666]
118618311  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
118637372  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
118626082  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
118632911  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
118639908  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A isoform X1 [KO:K07151] [EC:2.4.99.18]
118635969  MAGT1; magnesium transporter protein 1 isoform X1 [KO:K19478]
118622596  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
118632580  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
118628304  TMEM258; transmembrane protein 258 [KO:K26502]
118633624  transmembrane protein 258-like [KO:K26502]
118630703  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
118624697  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
118618754  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
118615833  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
118628326  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
118629806  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
118617653  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
118642643  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
118622540  DNAJC3; dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
118625564  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
118624183  HSP90B1; endoplasmin [KO:K09487]
118627530  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:3.2.1.207]
118615418  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
118644017  CANX; calnexin isoform X1 [KO:K08054]
118640726  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
118616065  CALR; calreticulin [KO:K08057]
118626866  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
118621197  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
118637416  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
118629766  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like isoform X1 [KO:K23741] [EC:3.2.1.209]
118615882  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
118618701  LMAN1; protein ERGIC-53 isoform X1 [KO:K10080]
118637361  LMAN1L; LOW QUALITY PROTEIN: protein ERGIC-53-like [KO:K10081]
118631043  PREB; prolactin regulatory element-binding protein [KO:K14003]
118615456  SAR1B; GTP-binding protein SAR1b isoform X1 [KO:K07953] [EC:3.6.5.-]
118632239  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
118633263  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
118618913  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
118632246  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
118618271  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
118626943  SEC23A; protein transport protein Sec23A [KO:K14006]
118626139  SEC24B; protein transport protein Sec24B isoform X1 [KO:K14007]
118615227  SEC24A; protein transport protein Sec24A [KO:K14007]
118616114  SEC24D; protein transport protein Sec24D [KO:K14007]
118632164  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
118625784  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
118622692  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
118633043  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
118619301  ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
118637080  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
118634480  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
118620368  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
118630837  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
118640186  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
118627202  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
118630894  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
118640554  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
118623617  OS9; protein OS-9 isoform X1 [KO:K10088]
118635982  LOW QUALITY PROTEIN: endoplasmic reticulum lectin 1-like [KO:K14008]
118630424  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
118626293  SSR1; translocon-associated protein subunit alpha isoform X1 [KO:K13249]
118637214  SSR2; translocon-associated protein subunit beta [KO:K13250]
118617188  SSR3; translocon-associated protein subunit gamma [KO:K13251]
118635800  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
118636596  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
118615817  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
118631602  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
118631556  DERL1; derlin-1 isoform X1 [KO:K11519]
118640108  DERL3; derlin-3 isoform X1 [KO:K13989]
118634843  DERL2; derlin-2 isoform X1 [KO:K13989]
118617388  LOW QUALITY PROTEIN: UBX domain-containing protein 4-like [KO:K24348]
118627914  UBXN1; UBX domain-containing protein 1 [KO:K24348]
118625435  UBXN4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
118630760  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
118616257  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
118622569  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
118618815  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
118628887  small VCP/p97-interacting protein isoform X1 [KO:K14014]
118629495  transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
118634086  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
118640075  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
118639973  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
118621036  HSPA1B; heat shock 70 kDa protein 1B [KO:K03283]
118621339  heat shock 70 kDa protein 1-like isoform X1 [KO:K03283]
118621340  HSPA1A; heat shock 70 kDa protein 1A [KO:K03283]
118629920  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
118639274  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
118616185  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
118625140  DNAJB2; dnaJ homolog subfamily B member 2 [KO:K09508]
118632386  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
118618305  DNAJC5; dnaJ homolog subfamily C member 5 isoform X1 [KO:K09525]
118631217  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
118631671  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
118627838  LOW QUALITY PROTEIN: heat shock protein HSP 90-beta-like [KO:K04079]
118634876  LOW QUALITY PROTEIN: heat shock protein HSP 90-beta-like [KO:K04079]
118635330  LOW QUALITY PROTEIN: heat shock protein HSP 90-beta-like [KO:K04079]
118621022  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
118637063  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
118637778  LOW QUALITY PROTEIN: heat shock protein HSP 90-beta-like [KO:K04079]
118615518  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
118624223  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
118629442  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
118621813  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
118638706  HSPBP1; hsp70-binding protein 1 [KO:K09562]
118617477  CRYAA; alpha-crystallin A chain isoform X1 [KO:K09541]
118628814  CRYAB; LOW QUALITY PROTEIN: alpha-crystallin B chain [KO:K09542]
118637088  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
118629763  PLAA; phospholipase A-2-activating protein [KO:K14018]
118630043  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
118615367  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
118628855  ubiquilin-3-like [KO:K04523]
118636618  UBQLN2; ubiquilin-2 [KO:K04523]
118629521  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
118637213  UBQLN4; ubiquilin-4 [KO:K04523]
118632919  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
118631855  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
118626198  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
118619213  LOW QUALITY PROTEIN: ubiquitin conjugation factor E4 B-like [KO:K10597] [EC:2.3.2.27]
118626254  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
118619537  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
118625163  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
118623538  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
118639353  PPP1R15A; protein phosphatase 1 regulatory subunit 15A isoform X1 [KO:K14019]
118623918  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
118619009  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
118637539  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
118621725  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
118619579  WFS1; wolframin [KO:K14020]
118638458  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
118635641  MBTPS2; membrane-bound transcription factor site-2 protease isoform X1 [KO:K07765] [EC:3.4.24.85]
118640291  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
118633993  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
118629846  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
118620900  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
118615560  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
118642157  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
118615122  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
118632456  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
118638701  BAX; apoptosis regulator BAX [KO:K02159]
118621850  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
118627889  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
118640556  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
118628924  caspase-12-like isoform X1 [KO:K04741] [EC:3.4.22.-]
118641679  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X1 [KO:K10661] [EC:2.3.2.27]
118621786  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
118643423  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
118634132  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
118617534  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X1 [KO:K04555] [EC:2.3.2.23]
118628039  SYVN1; E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
118621179  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X1 [KO:K10666] [EC:2.3.2.27]
118640167  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
118640842  SELENOS; selenoprotein S isoform X1 [KO:K14025]
118618540  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
118630519  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
118639046  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
118638422  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
118641703  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
118640865  LOW QUALITY PROTEIN: ubiquitin-conjugating enzyme E2 D3-like [KO:K06689] [EC:2.3.2.23]
118635861  ubiquitin-conjugating enzyme E2 D2B-like [KO:K06689] [EC:2.3.2.23]
118635956  ubiquitin-conjugating enzyme E2 4-like [KO:K06689] [EC:2.3.2.23]
118635965  ubiquitin-conjugating enzyme E2 4-like [KO:K06689] [EC:2.3.2.23]
118636090  ubiquitin-conjugating enzyme E2 D4-like [KO:K06689] [EC:2.3.2.23]
118636093  ubiquitin-conjugating enzyme E2 2-like [KO:K06689] [EC:2.3.2.23]
118636094  ubiquitin-conjugating enzyme E2 2-like [KO:K06689] [EC:2.3.2.23]
118636097  ubiquitin-conjugating enzyme E2 2-like [KO:K06689] [EC:2.3.2.23]
118636211  ubiquitin-conjugating enzyme E2 D2B-like [KO:K06689] [EC:2.3.2.23]
118636685  ubiquitin-conjugating enzyme E2 D4-like [KO:K06689] [EC:2.3.2.23]
118615169  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
118615188  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
118632189  LOW QUALITY PROTEIN: ubiquitin-conjugating enzyme E2 D3-like [KO:K06689] [EC:2.3.2.23]
118632553  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
118621965  PRKN; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
118618357  E3 ubiquitin-protein ligase RBX1-like [KO:K03868] [EC:2.3.2.32]
118624414  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
118619799  CUL1; cullin-1 [KO:K03347]
118621712  S-phase kinase-associated protein 1-like [KO:K03094]
118615636  SKP1; S-phase kinase-associated protein 1 isoform X1 [KO:K03094]
118638347  S-phase kinase-associated protein 1-like [KO:K03094]
118626880  FBXO2; F-box only protein 2 [KO:K10099]
118626454  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
mmf00510  N-Glycan biosynthesis
mmf03050  Proteasome
mmf03060  Protein export
mmf04210  Apoptosis
mmf04930  Type II diabetes mellitus
mmf05010  Alzheimer disease
mmf05012  Parkinson disease
mmf05020  Prion disease
KO pathway
ko04141   
LinkDB

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