KEGG   PATHWAY: mlf04141
Entry
mlf04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Myotis lucifugus (little brown 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
mlf04141  Protein processing in endoplasmic reticulum
mlf04141

Other DBs
GO: 0030433 0034976
Organism
Myotis lucifugus (little brown bat) [GN:mlf]
Gene
102420054  SEC61A2; LOW QUALITY PROTEIN: protein transport protein Sec61 subunit alpha [KO:K10956]
102426711  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
102432558  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
102418238  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
102421661  SEC62; translocation protein SEC62 isoform X1 [KO:K12275]
102432561  SEC63; translocation protein SEC63 homolog isoform X1 [KO:K09540]
102429503  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
102428695  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
102429327  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
102433681  DDOST; LOW QUALITY PROTEIN: dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
102419069  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
102437127  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
102419862  MAGT1; magnesium transporter protein 1 isoform X1 [KO:K19478]
102433387  TUSC3; tumor suppressor candidate 3 [KO:K19478]
102428320  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
102435013  TMEM258; transmembrane protein 258 isoform X1 [KO:K26502]
102429020  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
102436888  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
102425014  RRBP1; ribosome-binding protein 1 [KO:K14000]
102437566  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
102420315  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
102433604  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
102434257  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
102419929  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
102435427  DNAJC3; dnaJ homolog subfamily C member 3 isoform X1 [KO:K09523]
102426658  DNAJC10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530] [EC:1.8.4.-]
102422639  HSP90B1; endoplasmin [KO:K09487]
102439836  GANAB; neutral alpha-glucosidase AB [KO:K05546] [EC:3.2.1.207]
102419985  PRKCSH; glucosidase 2 subunit beta [KO:K08288]
102442616  CANX; calnexin isoform X1 [KO:K08054]
102433278  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
102438377  CALR; calreticulin [KO:K08057]
102425168  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X1 [KO:K01230] [EC:3.2.1.113]
102438711  MAN1A1; LOW QUALITY PROTEIN: mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
102437130  MAN1C1; LOW QUALITY PROTEIN: mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
102439240  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:3.2.1.209]
102421925  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
102440880  LMAN1; protein ERGIC-53 [KO:K10080]
102438443  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
102428767  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
102438239  SAR1A; GTP-binding protein SAR1a isoform X1 [KO:K07953] [EC:3.6.5.-]
102440448  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
102426667  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
102427684  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
102441650  SEC23A; protein transport protein Sec23A isoform X2 [KO:K14006]
102428657  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
102439623  SEC24D; protein transport protein Sec24D [KO:K14007]
102428451  SEC24A; protein transport protein Sec24A [KO:K14007]
102426539  SEC24B; protein transport protein Sec24B isoform X5 [KO:K14007]
102439419  SEC24C; protein transport protein Sec24C isoform X2 [KO:K14007]
102439642  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
102417777  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
102431556  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
102434914  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
102419915  EDEM3; LOW QUALITY PROTEIN: ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
102430298  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
102418654  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
102430559  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
102441453  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
102420298  TXNDC5; thioredoxin domain-containing protein 5 isoform X2 [KO:K13984] [EC:5.3.4.1]
102441759  ERO1A; ERO1-like protein alpha isoform X1 [KO:K10950] [EC:1.8.4.-]
102428059  LOW QUALITY PROTEIN: ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
102432681  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
102432639  OS9; protein OS-9 isoform X1 [KO:K10088]
102423842  ERLEC1; endoplasmic reticulum lectin 1 isoform X1 [KO:K14008]
102442590  translocon-associated protein subunit alpha [KO:K13249]
102422344  SSR1; translocon-associated protein subunit alpha [KO:K13249]
102417208  SSR2; translocon-associated protein subunit beta isoform X1 [KO:K13250]
102437187  SSR3; translocon-associated protein subunit gamma [KO:K13251]
102423760  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
102426059  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
102440668  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
102421949  DERL1; derlin-1 [KO:K11519]
102440425  DERL3; derlin-3 isoform X1 [KO:K13989]
102436411  DERL2; derlin-2 isoform X1 [KO:K13989]
102429231  UBXN4; UBX domain-containing protein 4 isoform X1 [KO:K24348]
102436435  UBXN1; UBX domain-containing protein 1 [KO:K24348]
102420342  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
102427513  UBXN6; UBX domain-containing protein 6 [KO:K14011]
102432812  UBXN8; UBX domain-containing protein 8 isoform X1 [KO:K24351]
102424181  NSFL1 cofactor p47 isoform X1 [KO:K14012]
102437697  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
102430640  SVIP; small VCP/p97-interacting protein [KO:K14014]
102419328  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
102442972  NPLOC4; LOW QUALITY PROTEIN: nuclear protein localization protein 4 homolog [KO:K14015]
102425753  ubiquitin recognition factor in ER-associated degradation protein 1-like [KO:K14016]
102426322  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
102425210  heat shock 70 kDa protein 1 [KO:K03283]
102425512  heat shock 70 kDa protein 1-like [KO:K03283]
102425826  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
102426882  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
102436597  DNAJA1; dnaJ homolog subfamily A member 1 isoform X1 [KO:K09502]
102425325  DNAJA4; LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 4 [KO:K09502]
102423795  LOW QUALITY PROTEIN: dnaJ homolog subfamily A member 1-like [KO:K09502]
102431859  DNAJA2; dnaJ homolog subfamily A member 2 isoform X1 [KO:K09503]
102421499  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
102431942  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
102422178  DNAJB12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518]
102421575  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
102441040  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X2 [KO:K09525]
102438162  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
102419327  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
102433810  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
102422444  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
102436494  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
102435361  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
102436672  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
102442343  HSPBP1; LOW QUALITY PROTEIN: hsp70-binding protein 1 [KO:K09562]
102431739  CRYAA; alpha-crystallin A chain isoform X2 [KO:K09541]
102433168  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
102432150  PLAA; phospholipase A-2-activating protein isoform X1 [KO:K14018]
102424278  RAD23B; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839]
102438687  RAD23A; UV excision repair protein RAD23 homolog A [KO:K10839]
102435654  UBQLN3; ubiquilin-3 [KO:K04523]
102424842  UBQLN2; ubiquilin-2 [KO:K04523]
102432661  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
102416897  UBQLN4; ubiquilin-4 [KO:K04523]
102435206  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
102435893  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
102429062  ubiquitin conjugation factor E4 B [KO:K10597] [EC:2.3.2.27]
102424368  UBE4B; ubiquitin conjugation factor E4 B isoform X9 [KO:K10597] [EC:2.3.2.27]
102433431  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
102428709  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
102437649  NFE2L2; nuclear factor erythroid 2-related factor 2 [KO:K05638]
102429560  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
102425426  PPP1R15A; protein phosphatase 1 regulatory subunit 15A isoform X1 [KO:K14019]
102440614  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
102432611  BCL2; LOW QUALITY PROTEIN: apoptosis regulator Bcl-2 [KO:K02161]
102426868  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
102428756  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
102442331  MBTPS1; membrane-bound transcription factor site-1 protease isoform X2 [KO:K08653] [EC:3.4.21.112]
111825283  YY2; LOW QUALITY PROTEIN: transcription factor YY2 [KO:K07765] [EC:3.4.24.85]
102435081  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
102424434  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
102419407  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
102439932  MAP3K5; LOW QUALITY PROTEIN: mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
102429748  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
102430582  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
102437455  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
102418507  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
102417074  BAX; apoptosis regulator BAX [KO:K02159]
102437326  BAK1; LOW QUALITY PROTEIN: bcl-2 homologous antagonist/killer [KO:K14021]
102430512  calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
102438585  LOW QUALITY PROTEIN: calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
102431309  LOW QUALITY PROTEIN: caspase-12-like [KO:K04741] [EC:3.4.22.-]
102443007  MARCH6; E3 ubiquitin-protein ligase MARCH6 isoform X1 [KO:K10661] [EC:2.3.2.27]
102425034  UBE2J1; ubiquitin-conjugating enzyme E2 J1 isoform X1 [KO:K10578] [EC:2.3.2.23]
102425338  UBE2J2; LOW QUALITY PROTEIN: ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
102429921  ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
102421740  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
102424647  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
102423647  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X4 [KO:K10601] [EC:2.3.2.27]
102432057  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:2.3.2.27]
102436743  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
102441177  E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:2.3.2.27]
102425947  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
102421651  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
102438790  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
102422374  LOW QUALITY PROTEIN: homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
102438292  AMFR; E3 ubiquitin-protein ligase AMFR isoform X1 [KO:K10636] [EC:2.3.2.36]
102440857  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
102436579  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X4 [KO:K06689] [EC:2.3.2.23]
102433341  UBE2D4; ubiquitin-conjugating enzyme E2 D4 isoform X1 [KO:K06689] [EC:2.3.2.23]
102432187  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
102422710  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X2 [KO:K06689] [EC:2.3.2.23]
102419305  ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
102439703  PRKN; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
102431403  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:2.3.2.32]
102421559  CUL1; cullin-1 isoform X1 [KO:K03347]
102432509  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
102436113  FBXO2; LOW QUALITY PROTEIN: F-box only protein 2 [KO:K10099]
102439075  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
mlf00510  N-Glycan biosynthesis
mlf03050  Proteasome
mlf03060  Protein export
mlf04210  Apoptosis
mlf04930  Type II diabetes mellitus
mlf05010  Alzheimer disease
mlf05012  Parkinson disease
mlf05020  Prion disease
KO pathway
ko04141   
LinkDB

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