KEGG   PATHWAY: ko04141Help
Entry
ko04141                     Pathway                                

Name
Protein processing in endoplasmic reticulum
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
BRITE hierarchy
Pathway map
Protein processing in endoplasmic reticulum
ko04141

All organismsOrtholog table
Module
M00382  
SCF-FBS complex [PATH:ko04141]
M00400  
p97-Ufd1-Npl4 complex [PATH:ko04141]
M00401  
Sec61 complex [PATH:ko04141]
M00402  
Translocon-associated protein (TRAP) complex [PATH:ko04141]
M00403  
HRD1/SEL1 ERAD complex [PATH:ko04141]
M00404  
COPII complex [PATH:ko04141]
Disease
H00078  
Frontotemporal lobar degeneration (FTLD)
H00221  
Combined deficiency of factors V and VIII (F5F8D)
H00344  
Leprosy
H00545  
Polycystic liver disease
H00594  
Distal muscular dystrophies
H00595  
Myofibrillar myopathies (MFM)
H00740  
Ichthyosis follicularis, alopecia, and photophobia syndrome
H00766  
Wolcott-Rallison syndrome
H00768  
Nonsyndromic autosomal recessive mental retardation (NS-ARMR)
H00854  
Wolfram syndrome (WFS)
H00917  
Congenital dyserythropoietic anemias (CDAs)
H00927  
Chylomicron retention disease (CRD)
H01105  
Cranio-lenticulo-sutural dysplasia (CLSD)
H01202  
Cataract
H01284  
Marinesco-Sjogren syndrome
Other DBs
Orthology
K10956  
protein transport protein SEC61 subunit alpha
K09481  
protein transport protein SEC61 subunit beta
K07342  
protein transport protein SEC61 subunit gamma and related proteins
K12275  
translocation protein SEC62
K09540  
translocation protein SEC63
K12666  
oligosaccharyltransferase complex subunit alpha (ribophorin I)
K12667  
oligosaccharyltransferase complex subunit delta (ribophorin II)
K12668  
oligosaccharyltransferase complex subunit epsilon
K12669  
oligosaccharyltransferase complex subunit gamma
K12670  
oligosaccharyltransferase complex subunit beta
K00730  
oligosaccharyl transferase complex subunit OST4
K12691  
oligosaccharyl transferase complex subunit OST5
K07151  
dolichyl-diphosphooligosaccharide--protein glycosyltransferase [EC:2.4.99.18]
K01228  
mannosyl-oligosaccharide glucosidase [EC:3.2.1.106]
K13999  
cytoskeleton-associated protein 4
K14000  
ribosome-binding protein 1
K14001  
nucleotide exchange factor SIL1
K09486  
hypoxia up-regulated 1
K09490  
heat shock 70kDa protein 5
K09517  
DnaJ homolog subfamily B member 11
K09521  
DnaJ homolog subfamily C member 1
K09523  
DnaJ homolog subfamily C member 3
K09530  
DnaJ homolog subfamily C member 10
K14002  
DnaJ-related protein SCJ1
K09487  
heat shock protein 90kDa beta
K05546  
alpha 1,3-glucosidase [EC:3.2.1.84]
K08288  
protein kinase C substrate 80K-H
K08054  
calnexin
K08056  
protein disulfide isomerase family A, member 3 [EC:5.3.4.1]
K08057  
calreticulin
K01230  
mannosyl-oligosaccharide alpha-1,2-mannosidase [EC:3.2.1.113]
K10082  
lectin, mannose-binding 2
K10080  
lectin, mannose-binding 1
K10081  
lectin, mannose-binding 1-like
K14003  
prolactin regulatory element-binding protein
K07953  
GTP-binding protein SAR1 [EC:3.6.5.-]
K14004  
protein transport protein SEC13
K14005  
protein transport protein SEC31
K14006  
protein transport protein SEC23
K14007  
protein transport protein SEC24
K11718  
UDP-glucose:glycoprotein glucosyltransferase [EC:2.4.1.-]
K10084  
ER degradation enhancer, mannosidase alpha-like 1
K10085  
ER degradation enhancer, mannosidase alpha-like 2
K10086  
ER degradation enhancer, mannosidase alpha-like 3
K09580  
protein disulfide-isomerase A1 [EC:5.3.4.1]
K09582  
protein disulfide-isomerase A4 [EC:5.3.4.1]
K09584  
protein disulfide-isomerase A6 [EC:5.3.4.1]
K13996  
protein disulfide-isomerase [EC:5.3.4.1]
K09586  
endoplasmic reticulum protein 29
K13984  
thioredoxin domain-containing protein 5
K10950  
ERO1-like protein alpha [EC:1.8.4.-]
K10976  
ERO1-like protein beta [EC:1.8.4.-]
K10088  
protein OS-9
K14008  
endoplasmic reticulum lectin 1
K13249  
translocon-associated protein subunit alpha
K13250  
translocon-associated protein subunit beta
K13251  
translocon-associated protein subunit gamma
K04571  
translocon-associated protein subunit delta
K14009  
B-cell receptor-associated protein 31
K14010  
translocating chain-associated membrane protein 1
K11519  
Derlin-1
K13989  
Derlin-2/3
K14011  
UBX domain-containing protein 6
K14012  
UBX domain-containing protein 1
K14013  
UBX domain-containing protein 2
K14014  
small VCP/p97-interacting protein
K13525  
transitional endoplasmic reticulum ATPase
K14015  
nuclear protein localization protein 4 homolog
K14016  
ubiquitin fusion degradation protein 1
K03283  
heat shock 70kDa protein 1/8
K09502  
DnaJ homolog subfamily A member 1
K09503  
DnaJ homolog subfamily A member 2
K09507  
DnaJ homolog subfamily B member 1
K09508  
DnaJ homolog subfamily B member 2
K09518  
DnaJ homolog subfamily B member 12
K09525  
DnaJ homolog subfamily C member 5
K04079  
molecular chaperone HtpG
K09485  
heat shock protein 110kDa
K09555  
BCL2-associated athanogene 1
K09556  
BCL2-associated athanogene 2
K09562  
hsp70-interacting protein
K14017  
HSP70 co-chaperone SNL1
K09541  
crystallin, alpha A
K09542  
crystallin, alpha B
K13993  
HSP20 family protein
K13719  
ubiquitin thioesterase OTU1 [EC:3.1.2.-]
K14018  
phospholipase A-2-activating protein
K10839  
UV excision repair protein RAD23
K04523  
ubiquilin
K01456  
peptide-N4-(N-acetyl-beta-glucosaminyl)asparagine amidase [EC:3.5.1.52]
K11863  
Ataxin-3 [EC:3.4.22.-]
K10597  
ubiquitin conjugation factor E4 B [EC:6.3.2.19]
K16194  
eukaryotic translation initiation factor 2-alpha kinase 1 [EC:2.7.11.1]
K16195  
eukaryotic translation initiation factor 2-alpha kinase 2 [EC:2.7.11.1]
K08860  
eukaryotic translation initiation factor 2-alpha kinase 3 [EC:2.7.11.1]
K16196  
eukaryotic translation initiation factor 2-alpha kinase 4 [EC:2.7.11.1]
K03237  
translation initiation factor 2 subunit 1
K05638  
nuclear factor erythroid 2-related factor 2
K04374  
cyclic AMP-dependent transcription factor ATF-4
K14019  
protein phosphatase 1 regulatory subunit 15A
K04452  
DNA damage-inducible transcript 3
K02161  
apoptosis regulator BCL-2
K09054  
cyclic AMP-dependent transcription factor ATF-6 alpha
K09049  
cyclic AMP-dependent transcription factor ATF-6 beta
K14020  
wolfamin
K08653  
membrane-bound transcription factor site-1 protease [EC:3.4.21.112]
K07765  
S2P endopeptidase [EC:3.4.24.85]
K09027  
X box-binding protein 1
K08852  
serine/threonine-protein kinase/endoribonuclease IRE1 [EC:3.1.26.- 2.7.11.1]
K03173  
TNF receptor-associated factor 2
K04426  
mitogen-activated protein kinase kinase kinase 5 [EC:2.7.11.25]
K04431  
mitogen-activated protein kinase kinase 7 [EC:2.7.12.2]
K04440  
c-Jun N-terminal kinase [EC:2.7.11.24]
K02159  
apoptosis regulator BAX
K14021  
Bcl-2 homologous antagonist/killer
K01367  
calpain-1 [EC:3.4.22.52]
K03853  
calpain-2 [EC:3.4.22.53]
K04741  
caspase 12 [EC:3.4.22.-]
K10661  
E3 ubiquitin-protein ligase MARCH6 [EC:6.3.2.19]
K10578  
ubiquitin-conjugating enzyme E2 J1 [EC:6.3.2.19]
K04554  
ubiquitin-conjugating enzyme E2 J2 [EC:6.3.2.19]
K10575  
ubiquitin-conjugating enzyme E2 G1 [EC:6.3.2.19]
K04555  
ubiquitin-conjugating enzyme E2 G2 [EC:6.3.2.19]
K14022  
coupling of ubiquitin conjugation to ER degradation protein 1
K14023  
ERAD-associated E3 ubiquitin-protein ligase component HRD3
K10601  
E3 ubiquitin-protein ligase synoviolin [EC:6.3.2.19]
K14024  
U1 SNP1-associating protein 1
K10666  
E3 ubiquitin-protein ligase RNF5 [EC:6.3.2.19]
K14025  
selenoprotein S
K14026  
SEL1 protein
K14027  
homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein
K10636  
autocrine motility factor receptor [EC:6.3.2.19]
K09561  
STIP1 homology and U-box containing protein 1 [EC:6.3.2.19]
K06689  
ubiquitin-conjugating enzyme E2 D/E [EC:6.3.2.19]
K04556  
parkin [EC:6.3.2.19]
K03868  
RING-box protein 1
K03347  
cullin 1
K03094  
S-phase kinase-associated protein 1
K10099  
F-box protein 2
K10100  
F-box protein 6
Compound
C00076  
Calcium cation
G00009  
 
G00010  
 
G00011  
 
G00012  
 
G10694  
 
Reference
  Authors
Naidoo N
  Title
ER and aging-Protein folding and the ER stress response.
  Journal
Ageing Res Rev 8:150-9 (2009)
Reference
  Authors
Malhotra JD, Kaufman RJ
  Title
The endoplasmic reticulum and the unfolded protein response.
  Journal
Semin Cell Dev Biol 18:716-31 (2007)
Reference
  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)
Reference
  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)
Reference
  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)

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