KEGG   PATHWAY: nni04141
Entry
nni04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Nipponia nippon (crested ibis)
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
nni04141  Protein processing in endoplasmic reticulum
nni04141

Other DBs
GO: 0030433 0034976
Organism
Nipponia nippon (crested ibis) [GN:nni]
Gene
104008647  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB [KO:K01230] [EC:3.2.1.113]
104008775  SYVN1; LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase synoviolin [KO:K10601] [EC:2.3.2.27]
104008776  [KO:K01367] [EC:3.4.22.52]
104008816  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
104008861  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
104008964  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
104008985  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
104008996  FBXO2; F-box only protein 2 [KO:K10099]
104008997  [KO:K10100]
104009507  DNAJB1; dnaJ homolog subfamily B member 1 [KO:K09507]
104009537  SEC23B; LOW QUALITY PROTEIN: protein transport protein Sec23B [KO:K14006]
104009543  RRBP1; LOW QUALITY PROTEIN: ribosome-binding protein 1 [KO:K14000]
104009593  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667]
104009670  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
104009759  UBXN4; UBX domain-containing protein 4 [KO:K24348]
104010090  DNAJB2; dnaJ homolog subfamily B member 2 [KO:K09508]
104010118  [KO:K08057]
104010225  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
104010239  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638]
104010410  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
104010649  UBE2G2; ubiquitin-conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
104010670  DNAJB11; dnaJ homolog subfamily B member 11 [KO:K09517]
104011142  SEC63; translocation protein SEC63 homolog [KO:K09540]
104011277  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
104011311  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086]
104011455  RNF185; E3 ubiquitin-protein ligase RNF185 isoform X1 [KO:K10666] [EC:2.3.2.27]
104011480  [KO:K13989]
104011591  SSR3; translocon-associated protein subunit gamma [KO:K13251]
104011624  ERO1LB; ERO1-like protein beta [KO:K10976] [EC:1.8.4.-]
104011758  UBQLN1; ubiquilin-1 [KO:K04523]
104011869  [KO:K09502]
104011971  SSR2; translocon-associated protein subunit beta [KO:K13250]
104011998  SEC24A; protein transport protein Sec24A [KO:K14007]
104011999  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
104012003  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
104012045  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668]
104012204  UBQLN4; ubiquilin-4 [KO:K04523]
104012402  [KO:K04523]
104013028  PDIA6; LOW QUALITY PROTEIN: protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
104013188  YOD1; ubiquitin thioesterase OTU1 [KO:K13719] [EC:3.1.2.-]
104013218  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
104013238  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
104013355  HSP90B1; endoplasmin [KO:K09487]
104013412  BAG1; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555]
104013456  SEC31B; protein transport protein Sec31B [KO:K14005]
104013502  SAR1A; GTP-binding protein SAR1a [KO:K07953] [EC:3.6.5.-]
104013506  DNAJB12; dnaJ homolog subfamily B member 12 [KO:K09518]
104013513  UBE2D1; ubiquitin-conjugating enzyme E2 D1 isoform X1 [KO:K06689] [EC:2.3.2.23]
104013662  PLAA; phospholipase A-2-activating protein [KO:K14018]
104013822  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
104013880  STT3B; LOW QUALITY PROTEIN: dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151] [EC:2.4.99.18]
104013960  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase [KO:K01456] [EC:3.5.1.52]
104014306  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
104014340  NPLOC4; nuclear protein localization protein 4 homolog [KO:K14015]
104014411  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
104014412  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
104014508  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
104014623  SEC61A2; protein transport protein Sec61 subunit alpha isoform 2 isoform X1 [KO:K10956]
104014646  UBXN2A; UBX domain-containing protein 2A [KO:K24349]
104015282  ERN2; serine/threonine-protein kinase/endoribonuclease IRE2 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
104015389  CUL1; cullin-1 isoform X1 [KO:K03347]
104015396  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
104015778  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
104015936  HSPA4L; heat shock 70 kDa protein 4L isoform X1 [KO:K09485]
104016001  PREB; prolactin regulatory element-binding protein [KO:K14003]
104016150  DERL2; derlin-2 [KO:K13989]
104016154  UBE2G1; ubiquitin-conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
104016217  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
104016242  HYOU1; LOW QUALITY PROTEIN: hypoxia up-regulated protein 1 [KO:K09486]
104016367  MBTPS1; membrane-bound transcription factor site-1 protease [KO:K08653] [EC:3.4.21.112]
104016377  AMFR; E3 ubiquitin-protein ligase AMFR [KO:K10636] [EC:2.3.2.36]
104016565  SIL1; nucleotide exchange factor SIL1 [KO:K14001]
104016627  HSPA8; heat shock cognate 71 kDa protein isoform X1 [KO:K03283]
104016655  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
104016947  EDEM1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084]
104017015  SEC13; LOW QUALITY PROTEIN: protein SEC13 homolog [KO:K14004]
104017170  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
104017223  VIMP; selenoprotein S [KO:K14025]
104017467  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027]
104017505  SEC24C; protein transport protein Sec24C isoform X1 [KO:K14007]
104017554  SSR4; translocon-associated protein subunit delta [KO:K04571]
104017726  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC [KO:K01230] [EC:3.2.1.113]
104018013  DERL1; derlin-1 [KO:K11519]
104018038  WFS1; wolframin [KO:K14020]
104018203  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
104018369  TRAF2; LOW QUALITY PROTEIN: TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
104018375  MAN1B1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741]
104018425  PARK2; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
104018438  CANX; LOW QUALITY PROTEIN: calnexin [KO:K08054]
104018444  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
104018496  [KO:K09525]
104018561  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
104018629  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
104018745  UBXN6; UBX domain-containing protein 6 [KO:K14011]
104018793  CALR3; calreticulin-3 [KO:K08057]
104018861  MARCH6; E3 ubiquitin-protein ligase MARCH6 [KO:K10661] [EC:2.3.2.27]
104019006  LMAN2; vesicular integral-membrane protein VIP36 [KO:K10082]
104019033  [KO:K09502]
104019050  CRYAB; alpha-crystallin B chain [KO:K09542]
104019133  SEC24D; LOW QUALITY PROTEIN: protein transport protein Sec24D [KO:K14007]
104019181  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
104019299  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
104019479  ERO1L; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
104019812  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
104019815  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
104019931  [KO:K14010]
104019981  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
104020032  DNAJC5; dnaJ homolog subfamily C member 5 [KO:K09525]
104020045  NSFL1C; NSFL1 cofactor p47 [KO:K14012]
104020071  UFD1L; ubiquitin fusion degradation protein 1 homolog isoform X1 [KO:K14016]
104020299  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
104020493  KRTCAP2; keratinocyte-associated protein 2 [KO:K28202]
104020510  OS9; protein OS-9 [KO:K10088]
104020586  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
104020749  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
104020831  TUSC3; tumor suppressor candidate 3 [KO:K19478]
104020849  CRYAA; alpha-crystallin A chain [KO:K09541]
104020907  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
104020971  LMAN1; protein ERGIC-53 [KO:K10080]
104020979  SEC23A; protein transport protein Sec23A [KO:K14006]
104021162  SEC24B; protein transport protein Sec24B [KO:K14007]
104021164  OSTC; oligosaccharyltransferase complex subunit OSTC isoform X1 [KO:K26501]
104021182  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X1 [KO:K06689] [EC:2.3.2.23]
104021477  [KO:K28202]
104021515  HSP90AB1; heat shock protein HSP 90-beta isoform X1 [KO:K04079]
104021518  CAPN11; calpain-11 [KO:K01367] [EC:3.4.22.52]
104021625  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
104021829  TXNDC5; thioredoxin domain-containing protein 5 [KO:K13984] [EC:5.3.4.1]
104021857  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
104021903  SSR1; translocon-associated protein subunit alpha [KO:K13249]
104021947  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
104022047  SEC62; translocation protein SEC62 [KO:K12275]
104022141  [KO:K06689] [EC:2.3.2.23]
104022214  ATXN3; ataxin-3 [KO:K11863] [EC:3.4.22.-]
104022468  ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
104022509  MBTPS2; membrane-bound transcription factor site-2 protease [KO:K07765] [EC:3.4.24.85]
104022637  DNAJA1; dnaJ homolog subfamily A member 1 [KO:K09502]
104022671  SEC61A1; protein transport protein Sec61 subunit alpha isoform 1 [KO:K10956]
104022676  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
104023010  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
104023205  MAGT1; magnesium transporter protein 1 [KO:K19478]
104023331  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
104023407  RAD23B; UV excision repair protein RAD23 homolog B [KO:K10839]
104023421  SEL1L; protein sel-1 homolog 1 [KO:K14026]
104023475  STUB1; LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
104023691  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
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
nni00510  N-Glycan biosynthesis
nni03050  Proteasome
nni03060  Protein export
nni04210  Apoptosis
KO pathway
ko04141   

DBGET integrated database retrieval system