KEGG   PATHWAY: hsa04141Help
Entry
hsa04141                    Pathway                                

Name
Protein processing in endoplasmic reticulum - Homo sapiens (human)
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
hsa04141

All organismsOrtholog table
Module
SCF-FBS complex [PATH:hsa04141]
p97-Ufd1-Npl4 complex [PATH:hsa04141]
Sec61 complex [PATH:hsa04141]
Translocon-associated protein (TRAP) complex [PATH:hsa04141]
HRD1/SEL1 ERAD complex [PATH:hsa04141]
COPII complex [PATH:hsa04141]
Disease
H00078  
Frontotemporal lobar degeneration (FTLD)
H00118  
Congenital disorders of glycosylation (CDG) type I
H00221  
Combined deficiency of factors V and VIII (F5F8D)
H00344  
Leprosy
H00409  
Type II diabetes mellitus
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
H01478  
Machado-Joseph disease
H01572  
Cole-Carpenter syndrome
Other DBs
Organism
Homo sapiens (human) [GN:hsa]
Gene
29927  
SEC61A1; Sec61 translocon alpha 1 subunit [KO:K10956]
55176  
SEC61A2; Sec61 translocon alpha 2 subunit [KO:K10956]
10952  
SEC61B; Sec61 translocon beta subunit [KO:K09481]
23480  
SEC61G; Sec61 translocon gamma subunit [KO:K07342]
7095  
SEC62; SEC62 homolog, preprotein translocation factor [KO:K12275]
11231  
SEC63; SEC63 homolog, protein translocation regulator [KO:K09540]
6184  
RPN1; ribophorin I [KO:K12666]
6185  
RPN2; ribophorin II [KO:K12667]
1603  
DAD1; defender against cell death 1 [KO:K12668]
7991  
TUSC3; tumor suppressor candidate 3 [KO:K12669]
1650  
DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase non-catalytic subunit [KO:K12670]
3703  
STT3A; STT3A, catalytic subunit of the oligosaccharyltransferase complex [KO:K07151] [EC:2.4.99.18]
201595  
STT3B; STT3B, catalytic subunit of the oligosaccharyltransferase complex [KO:K07151] [EC:2.4.99.18]
7841  
MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
10970  
CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
6238  
RRBP1; ribosome binding protein 1 [KO:K14000]
64374  
SIL1; SIL1 nucleotide exchange factor [KO:K14001]
10525  
HYOU1; hypoxia up-regulated 1 [KO:K09486]
3309  
HSPA5; heat shock protein family A (Hsp70) member 5 [KO:K09490]
51726  
DNAJB11; DnaJ heat shock protein family (Hsp40) member B11 [KO:K09517]
64215  
DNAJC1; DnaJ heat shock protein family (Hsp40) member C1 [KO:K09521]
5611  
DNAJC3; DnaJ heat shock protein family (Hsp40) member C3 [KO:K09523]
54431  
DNAJC10; DnaJ heat shock protein family (Hsp40) member C10 [KO:K09530]
7184  
HSP90B1; heat shock protein 90kDa beta family member 1 [KO:K09487]
23193  
GANAB; glucosidase II alpha subunit [KO:K05546] [EC:3.2.1.84]
5589  
PRKCSH; protein kinase C substrate 80K-H [KO:K08288]
821  
CANX; calnexin [KO:K08054]
2923  
PDIA3; protein disulfide isomerase family A member 3 [KO:K08056] [EC:5.3.4.1]
811  
CALR; calreticulin [KO:K08057]
11253  
MAN1B1; mannosidase alpha class 1B member 1 [KO:K01230] [EC:3.2.1.113]
10905  
MAN1A2; mannosidase alpha class 1A member 2 [KO:K01230] [EC:3.2.1.113]
57134  
MAN1C1; mannosidase alpha class 1C member 1 [KO:K01230] [EC:3.2.1.113]
4121  
MAN1A1; mannosidase alpha class 1A member 1 [KO:K01230] [EC:3.2.1.113]
10960  
LMAN2; lectin, mannose binding 2 [KO:K10082]
3998  
LMAN1; lectin, mannose binding 1 [KO:K10080]
79748  
LMAN1L; lectin, mannose binding 1 like [KO:K10081]
10113  
PREB; prolactin regulatory element binding [KO:K14003]
56681  
SAR1A; secretion associated Ras related GTPase 1A [KO:K07953] [EC:3.6.5.-]
51128  
SAR1B; secretion associated Ras related GTPase 1B [KO:K07953] [EC:3.6.5.-]
6396  
SEC13; SEC13 homolog, nuclear pore and COPII coat complex component [KO:K14004]
22872  
SEC31A; SEC31 homolog A, COPII coat complex component [KO:K14005]
25956  
SEC31B; SEC31 homolog B, COPII coat complex component [KO:K14005]
10483  
SEC23B; Sec23 homolog B, coat complex II component [KO:K14006]
10484  
SEC23A; Sec23 homolog A, coat complex II component [KO:K14006]
10427  
SEC24B; SEC24 homolog B, COPII coat complex component [KO:K14007]
10802  
SEC24A; SEC24 homolog A, COPII coat complex component [KO:K14007]
9632  
SEC24C; SEC24 homolog C, COPII coat complex component [KO:K14007]
9871  
SEC24D; SEC24 homolog D, COPII coat complex component [KO:K14007]
55757  
UGGT2; UDP-glucose glycoprotein glucosyltransferase 2 [KO:K11718] [EC:2.4.1.-]
56886  
UGGT1; UDP-glucose glycoprotein glucosyltransferase 1 [KO:K11718] [EC:2.4.1.-]
9695  
EDEM1; ER degradation enhancing alpha-mannosidase like protein 1 [KO:K10084]
55741  
EDEM2; ER degradation enhancing alpha-mannosidase like protein 2 [KO:K10085]
80267  
EDEM3; ER degradation enhancing alpha-mannosidase like protein 3 [KO:K10086]
5034  
P4HB; prolyl 4-hydroxylase subunit beta [KO:K09580] [EC:5.3.4.1]
9601  
PDIA4; protein disulfide isomerase family A member 4 [KO:K09582] [EC:5.3.4.1]
10130  
PDIA6; protein disulfide isomerase family A member 6 [KO:K09584] [EC:5.3.4.1]
10961  
ERP29; endoplasmic reticulum protein 29 [KO:K09586]
81567  
TXNDC5; thioredoxin domain containing 5 [KO:K13984]
30001  
ERO1A; endoplasmic reticulum oxidoreductase alpha [KO:K10950] [EC:1.8.4.-]
56605  
ERO1B; endoplasmic reticulum oxidoreductase beta [KO:K10976] [EC:1.8.4.-]
10956  
OS9; OS9, endoplasmic reticulum lectin [KO:K10088]
27248  
ERLEC1; endoplasmic reticulum lectin 1 [KO:K14008]
6745  
SSR1; signal sequence receptor subunit 1 [KO:K13249]
6746  
SSR2; signal sequence receptor subunit 2 [KO:K13250]
6747  
SSR3; signal sequence receptor subunit 3 [KO:K13251]
6748  
SSR4; signal sequence receptor subunit 4 [KO:K04571]
10134  
BCAP31; B-cell receptor-associated protein 31 [KO:K14009]
23471  
TRAM1; translocation associated membrane protein 1 [KO:K14010]
79139  
DERL1; derlin 1 [KO:K11519]
51009  
DERL2; derlin 2 [KO:K13989]
91319  
DERL3; derlin 3 [KO:K13989]
80700  
UBXN6; UBX domain protein 6 [KO:K14011]
55968  
NSFL1C; NSFL1 cofactor [KO:K14012]
258010  
SVIP; small VCP/p97-interacting protein [KO:K14014]
7415  
VCP; valosin containing protein [KO:K13525]
55666  
NPLOC4; NPL4 homolog, ubiquitin recognition factor [KO:K14015]
7353  
UFD1L; ubiquitin fusion degradation 1 like (yeast) [KO:K14016]
3312  
HSPA8; heat shock protein family A (Hsp70) member 8 [KO:K03283]
3303  
HSPA1A; heat shock protein family A (Hsp70) member 1A [KO:K03283]
3306  
HSPA2; heat shock protein family A (Hsp70) member 2 [KO:K03283]
3305  
HSPA1L; heat shock protein family A (Hsp70) member 1 like [KO:K03283]
3304  
HSPA1B; heat shock protein family A (Hsp70) member 1B [KO:K03283]
3310  
HSPA6; heat shock protein family A (Hsp70) member 6 [KO:K03283]
3301  
DNAJA1; DnaJ heat shock protein family (Hsp40) member A1 [KO:K09502]
10294  
DNAJA2; DnaJ heat shock protein family (Hsp40) member A2 [KO:K09503]
3337  
DNAJB1; DnaJ heat shock protein family (Hsp40) member B1 [KO:K09507]
3300  
DNAJB2; DnaJ heat shock protein family (Hsp40) member B2 [KO:K09508]
54788  
DNAJB12; DnaJ heat shock protein family (Hsp40) member B12 [KO:K09518]
80331  
DNAJC5; DnaJ heat shock protein family (Hsp40) member C5 [KO:K09525]
85479  
DNAJC5B; DnaJ heat shock protein family (Hsp40) member C5 beta [KO:K09525]
285126  
DNAJC5G; DnaJ heat shock protein family (Hsp40) member C5 gamma [KO:K09525]
3320  
HSP90AA1; heat shock protein 90kDa alpha family class A member 1 [KO:K04079]
3326  
HSP90AB1; heat shock protein 90kDa alpha family class B member 1 [KO:K04079]
10808  
HSPH1; heat shock protein family H (Hsp110) member 1 [KO:K09485]
22824  
HSPA4L; heat shock protein family A (Hsp70) member 4 like [KO:K09485]
573  
BAG1; BCL2 associated athanogene 1 [KO:K09555]
9532  
BAG2; BCL2 associated athanogene 2 [KO:K09556]
23640  
HSPBP1; HSPA (heat shock 70kDa) binding protein, cytoplasmic cochaperone 1 [KO:K09562]
1409  
CRYAA; crystallin alpha A [KO:K09541]
CRYAA; alpha-crystallin A chain [KO:K09541]
1410  
CRYAB; crystallin alpha B [KO:K09542]
55432  
YOD1; YOD1 deubiquitinase [KO:K13719] [EC:3.1.2.-]
9373  
PLAA; phospholipase A2 activating protein [KO:K14018]
5887  
RAD23B; RAD23 homolog B, nucleotide excision repair protein [KO:K10839]
5886  
RAD23A; RAD23 homolog A, nucleotide excision repair protein [KO:K10839]
29979  
UBQLN1; ubiquilin 1 [KO:K04523]
29978  
UBQLN2; ubiquilin 2 [KO:K04523]
50613  
UBQLN3; ubiquilin 3 [KO:K04523]
56893  
UBQLN4; ubiquilin 4 [KO:K04523]
143630  
UBQLNL; ubiquilin-like [KO:K04523]
55768  
NGLY1; N-glycanase 1 [KO:K01456] [EC:3.5.1.52]
4287  
ATXN3; ataxin 3 [KO:K11863] [EC:3.4.22.-]
92552  
ATXN3L; ataxin 3 like [KO:K11863] [EC:3.4.22.-]
10277  
UBE4B; ubiquitination factor E4B [KO:K10597] [EC:2.3.2.27]
27102  
EIF2AK1; eukaryotic translation initiation factor 2 alpha kinase 1 [KO:K16194] [EC:2.7.11.1]
5610  
EIF2AK2; eukaryotic translation initiation factor 2 alpha kinase 2 [KO:K16195] [EC:2.7.11.1]
9451  
EIF2AK3; eukaryotic translation initiation factor 2 alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
440275  
EIF2AK4; eukaryotic translation initiation factor 2 alpha kinase 4 [KO:K16196] [EC:2.7.11.1]
1965  
EIF2S1; eukaryotic translation initiation factor 2 subunit alpha [KO:K03237]
4780  
NFE2L2; nuclear factor, erythroid 2 like 2 [KO:K05638]
468  
ATF4; activating transcription factor 4 [KO:K04374]
23645  
PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
1649  
DDIT3; DNA damage inducible transcript 3 [KO:K04452]
596  
BCL2; B-cell CLL/lymphoma 2 [KO:K02161]
22926  
ATF6; activating transcription factor 6 [KO:K09054]
1388  
ATF6B; activating transcription factor 6 beta [KO:K09049]
7466  
WFS1; wolframin ER transmembrane glycoprotein [KO:K14020]
8720  
MBTPS1; membrane bound transcription factor peptidase, site 1 [KO:K08653] [EC:3.4.21.112]
51360  
MBTPS2; membrane bound transcription factor peptidase, site 2 [KO:K07765] [EC:3.4.24.85]
7494  
XBP1; X-box binding protein 1 [KO:K09027]
2081  
ERN1; endoplasmic reticulum to nucleus signaling 1 [KO:K08852] [EC:3.1.26.- 2.7.11.1]
7186  
TRAF2; TNF receptor associated factor 2 [KO:K03173] [EC:2.3.2.27]
4217  
MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
5609  
MAP2K7; mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
5599  
MAPK8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
5602  
MAPK10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
5601  
MAPK9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
581  
BAX; BCL2 associated X protein [KO:K02159]
578  
BAK1; BCL2 antagonist/killer 1 [KO:K14021]
823  
CAPN1; calpain 1 [KO:K01367] [EC:3.4.22.52]
824  
CAPN2; calpain 2 [KO:K03853] [EC:3.4.22.53]
CASP12; caspase 12 (gene/pseudogene) [KO:K04741] [EC:3.4.22.-]
10299  
MARCH6; membrane associated ring-CH-type finger 6 [KO:K10661] [EC:2.3.2.27]
51465  
UBE2J1; ubiquitin conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
118424  
UBE2J2; ubiquitin conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
7326  
UBE2G1; ubiquitin conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
7327  
UBE2G2; ubiquitin conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
84447  
SYVN1; synoviolin 1 [KO:K10601] [EC:2.3.2.27]
6048  
RNF5; ring finger protein 5 [KO:K10666] [EC:2.3.2.27]
91445  
RNF185; ring finger protein 185 [KO:K10666] [EC:2.3.2.27]
55829  
VIMP; VCP interacting membrane selenoprotein [KO:K14025]
6400  
SEL1L; SEL1L ERAD E3 ligase adaptor subunit [KO:K14026]
80343  
SEL1L2; SEL1L2 ERAD E3 ligase adaptor subunit [KO:K14026]
9709  
HERPUD1; homocysteine inducible ER protein with ubiquitin like domain 1 [KO:K14027]
267  
AMFR; autocrine motility factor receptor [KO:K10636] [EC:2.3.2.27]
10273  
STUB1; STIP1 homology and U-box containing protein 1 [KO:K09561] [EC:2.3.2.27]
51619  
UBE2D4; ubiquitin conjugating enzyme E2 D4 (putative) [KO:K06689] [EC:2.3.2.23]
7321  
UBE2D1; ubiquitin conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
7322  
UBE2D2; ubiquitin conjugating enzyme E2 D2 [KO:K06689] [EC:2.3.2.23]
7323  
UBE2D3; ubiquitin conjugating enzyme E2 D3 [KO:K06689] [EC:2.3.2.23]
5071  
PARK2; parkin RBR E3 ubiquitin protein ligase [KO:K04556] [EC:2.3.2.27]
9978  
RBX1; ring-box 1 [KO:K03868]
8454  
CUL1; cullin 1 [KO:K03347]
6500  
SKP1; S-phase kinase-associated protein 1 [KO:K03094]
26232  
FBXO2; F-box protein 2 [KO:K10099]
26270  
FBXO6; F-box protein 6 [KO:K10100]
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)
KO pathway
 

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