KEGG   PATHWAY: mna04141
mna04141                    Pathway                                
Protein processing in endoplasmic reticulum - Miniopterus natalensis (Natal long-fingered bat)
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.
Genetic Information Processing; Folding, sorting and degradation
Pathway map
mna04141  Protein processing in endoplasmic reticulum

Other DBs
GO: 0030433 0034976
Miniopterus natalensis (Natal long-fingered bat) [GN:mna]
107525213  SEC61A1; protein transport protein Sec61 subunit alpha [KO:K10956]
107530245  SEC61A2; protein transport protein Sec61 subunit alpha [KO:K10956]
107545034  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
107525995  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
107529544  SEC62; translocation protein SEC62 isoform X1 [KO:K12275]
107540708  SEC63; translocation protein SEC63 homolog [KO:K09540]
107525217  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
107542111  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667]
107531686  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 isoform X1 [KO:K12668]
107534078  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K12669]
107538427  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670]
107530589  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:]
107526382  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:]
107529255  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:]
107545165  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
107536340  RRBP1; ribosome-binding protein 1 [KO:K14000]
107530132  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
107533100  HYOU1; hypoxia up-regulated protein 1 isoform X1 [KO:K09486]
107535485  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:]
107527309  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
107546328  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
107545897  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
107535914  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
107545155  HSP90B1; endoplasmin [KO:K09487]
107543105  GANAB; neutral alpha-glucosidase AB isoform X1 [KO:K05546] [EC:]
107533274  PRKCSH; glucosidase 2 subunit beta [KO:K08288]
107528428  CANX; calnexin [KO:K08054]
107535925  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:]
107541519  CALR; calreticulin [KO:K08057]
107538021  calreticulin-like [KO:K08057]
107539790  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:]
107545314  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:]
107526302  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:]
107536151  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741] [EC:]
107529775  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
107545441  LMAN1; protein ERGIC-53 isoform X1 [KO:K10080]
107535203  LMAN1L; LOW QUALITY PROTEIN: protein ERGIC-53-like [KO:K10081]
107534342  PREB; prolactin regulatory element-binding protein isoform X1 [KO:K14003]
107525390  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
107526026  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
107534741  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
107542655  SEC31B; protein transport protein Sec31B [KO:K14005]
107537137  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
107534632  SEC23A; protein transport protein Sec23A [KO:K14006]
107536332  SEC23B; protein transport protein Sec23B [KO:K14006]
107525387  SEC24A; protein transport protein Sec24A isoform X1 [KO:K14007]
107534371  SEC24B; protein transport protein Sec24B [KO:K14007]
107527847  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
107545835  SEC24C; protein transport protein Sec24C [KO:K14007]
107545896  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
107528573  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
107529152  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
107537205  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085]
107546284  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086]
107539045  P4HB; protein disulfide-isomerase [KO:K09580] [EC:]
107523800  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:]
107543536  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:]
107529612  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
107529127  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984]
107525871  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
107527204  ERO1B; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
107546217  OS9; protein OS-9 isoform X1 [KO:K10088]
107544319  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
107529121  SSR1; translocon-associated protein subunit alpha [KO:K13249]
107544692  SSR3; translocon-associated protein subunit gamma [KO:K13251]
107538804  SSR4; translocon-associated protein subunit delta isoform X1 [KO:K04571]
107538811  BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
107539557  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
107533169  DERL1; derlin-1 [KO:K11519]
107528049  DERL2; derlin-2 isoform X1 [KO:K13989]
107530530  DERL3; derlin-3 [KO:K13989]
107532005  UBXN4; UBX domain-containing protein 4 [KO:K24348]
107543110  UBXN1; UBX domain-containing protein 1 [KO:K24348]
107533965  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
107525648  UBXN6; UBX domain-containing protein 6 isoform X1 [KO:K14011]
107534102  UBXN8; UBX domain-containing protein 8 [KO:K24351]
107544437  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
107544988  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
107539052  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
107530559  UFD1L; ubiquitin fusion degradation protein 1 homolog [KO:K14016]
107539014  LOW QUALITY PROTEIN: heat shock 70 kDa protein 1-like [KO:K03283]
107538950  heat shock 70 kDa protein 1 [KO:K03283]
107540605  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
107545657  HSPA2; heat shock-related 70 kDa protein 2 [KO:K03283]
107545008  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
107535867  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
107542378  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
107524782  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
107545846  DNAJB12; LOW QUALITY PROTEIN: dnaJ homolog subfamily B member 12 [KO:K09518]
107532149  DNAJC5B; dnaJ homolog subfamily C member 5B [KO:K09525]
107528911  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
107534825  DNAJC5G; dnaJ homolog subfamily C member 5G [KO:K09525]
107536915  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
107536502  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
107529803  HSPA4L; heat shock 70 kDa protein 4L [KO:K09485]
107537535  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
107544865  BAG1; BAG family molecular chaperone regulator 1 [KO:K09555]
107525144  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
107541319  HSPBP1; hsp70-binding protein 1 [KO:K09562]
107541250  CRYAA; alpha-crystallin A chain isoform X1 [KO:K09541]
107531294  CRYAB; alpha-crystallin B chain [KO:K09542]
107538947  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
107543089  PLAA; phospholipase A-2-activating protein [KO:K14018]
107541513  RAD23A; UV excision repair protein RAD23 homolog A [KO:K10839]
107537666  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
107543839  ubiquilin-4 isoform X1 [KO:K04523]
107538694  ubiquilin-3-like [KO:K04523]
107537267  UBQLN2; ubiquilin-2 [KO:K04523]
107544952  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
107526368  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:]
107531595  ATXN3; ataxin-3 isoform X1 [KO:K11863] [EC:3.4.22.-]
107538400  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:]
107533921  EIF2AK1; eukaryotic translation initiation factor 2-alpha kinase 1 [KO:K16194] [EC:]
107541819  EIF2AK2; interferon-induced, double-stranded RNA-activated protein kinase isoform X1 [KO:K16195] [EC:]
107528720  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:]
107542988  EIF2AK4; eIF-2-alpha kinase GCN2 [KO:K16196] [EC:]
107545674  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 isoform X1 [KO:K03237]
107533987  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
107531265  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
107531903  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
107546204  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
107545431  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
107535122  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
107539006  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
107524058  WFS1; wolframin [KO:K14020]
107524966  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:]
107526794  MBTPS2; LOW QUALITY PROTEIN: membrane-bound transcription factor site-2 protease [KO:K07765] [EC:]
107526248  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
107530680  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC: 3.1.26.-]
107536208  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:]
107530984  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:]
107529906  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:]
107537133  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:]
107527953  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:]
107528432  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:]
107531901  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
107541604  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
107533638  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:]
107543584  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:]
107532383  caspase-12-like isoform X1 [KO:K04741] [EC:3.4.22.-]
107540549  MARCH6; E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:]
107533438  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:]
107543702  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:]
107528039  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:]
107537476  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:]
107533639  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X1 [KO:K10601] [EC:]
107526174  RNF185; E3 ubiquitin-protein ligase RNF185 [KO:K10666] [EC:]
107538994  RNF5; E3 ubiquitin-protein ligase RNF5 [KO:K10666] [EC:]
107527989  VIMP; selenoprotein S [KO:K14025]
107542633  SEL1L; protein sel-1 homolog 1 [KO:K14026]
107536276  SEL1L2; protein sel-1 homolog 2 [KO:K14026]
107543513  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027]
107543368  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:]
107543221  STUB1; E3 ubiquitin-protein ligase CHIP isoform X1 [KO:K09561] [EC:]
107527552  ubiquitin-conjugating enzyme E2 2-like [KO:K06689] [EC:]
107527562  ubiquitin-conjugating enzyme E2-17 kDa-like [KO:K06689] [EC:]
107527563  ubiquitin-conjugating enzyme E2-17 kDa-like isoform X1 [KO:K06689] [EC:]
107542505  ubiquitin-conjugating enzyme E2 D2B-like [KO:K06689] [EC:]
107545515  UBE2D3; ubiquitin-conjugating enzyme E2 D3 [KO:K06689] [EC:]
107530137  UBE2D2; ubiquitin-conjugating enzyme E2 D2 [KO:K06689] [EC:]
107526862  ubiquitin-conjugating enzyme E2 D3-like [KO:K06689] [EC:]
107543351  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:]
107531636  ubiquitin-conjugating enzyme E2 D2B-like [KO:K06689] [EC:]
107542057  UBE2D4; LOW QUALITY PROTEIN: ubiquitin-conjugating enzyme E2 D4 [KO:K06689] [EC:]
107535732  PARK2; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:]
107531277  RBX1; E3 ubiquitin-protein ligase RBX1 [KO:K03868] [EC:]
107523767  CUL1; cullin-1 isoform X1 [KO:K03347]
107525395  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
107540989  FBXO2; F-box only protein 2 [KO:K10099]
107540999  FBXO6; F-box only protein 6 [KO:K10100]
C00076  Calcium cation
Naidoo N
ER and aging-Protein folding and the ER stress response.
Ageing Res Rev 8:150-9 (2009)
Malhotra JD, Kaufman RJ
The endoplasmic reticulum and the unfolded protein response.
Semin Cell Dev Biol 18:716-31 (2007)
Maattanen P, Gehring K, Bergeron JJ, Thomas DY
Protein quality control in the ER: the recognition of misfolded proteins.
Semin Cell Dev Biol 21:500-11 (2010)
Stolz A, Wolf DH
Endoplasmic reticulum associated protein degradation: a chaperone assisted journey to hell.
Biochim Biophys Acta 1803:694-705 (2010)
Dejgaard K, Theberge JF, Heath-Engel H, Chevet E, Tremblay ML, Thomas DY
Organization of the Sec61 translocon, studied by high resolution native electrophoresis.
J Proteome Res 9:1763-71 (2010)
mna00510  N-Glycan biosynthesis
mna03050  Proteasome
mna03060  Protein export
mna04210  Apoptosis
mna04930  Type II diabetes mellitus
mna05010  Alzheimer disease
mna05012  Parkinson disease
mna05020  Prion disease
KO pathway

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