Entry
Name
Protein processing in endoplasmic reticulum - Mastomys coucha (southern multimammate mouse)
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
mcoc04141 Protein processing in endoplasmic reticulum
Ortholog table
Other DBs
Organism
Mastomys coucha (southern multimammate mouse) [GN:
mcoc ]
Gene
116099225 Sec61a1; protein transport protein Sec61 subunit alpha [KO:K10956 ]
116082463 Sec61a2; protein transport protein Sec61 subunit alpha [KO:K10956 ]
116070121 Sec61g; LOW QUALITY PROTEIN: protein transport protein Sec61 subunit gamma [KO:K07342 ]
116075444 Sec63; translocation protein SEC63 homolog isoform X1 [KO:K09540 ]
116099218 Rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666 ]
116091286 Rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667 ]
116084297 Dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668 ]
116096900 Ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670 ]
116073583 Stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151 ] [EC:2.4.99.18 ]
116071916 Stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A isoform X1 [KO:K07151 ] [EC:2.4.99.18 ]
116090376 Dnajc10; dnaJ homolog subfamily C member 10 [KO:K09530 ] [EC:1.8.4.-]
116089811 Man1b1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741 ] [EC:3.2.1.209 ]
116080051 Preb; prolactin regulatory element-binding protein isoform X1 [KO:K14003 ]
116084234 Uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718 ] [EC:2.4.1.-]
116088291 Uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
116099366 Edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084 ]
116091245 Edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085 ]
116100905 Edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
116081487 Ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249 ]
116086016 Ssr4; translocon-associated protein subunit delta isoform X1 [KO:K04571 ]
116093742 Tram1l1; translocating chain-associated membrane protein 1-like 1 [KO:K14010 ]
116088185 Tram1; translocating chain-associated membrane protein 1 [KO:K14010 ]
116079979 Ubxn2a; LOW QUALITY PROTEIN: UBX domain-containing protein 2A [KO:K24349 ]
116095875 Vcp; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525 ]
116078806 Nploc4; nuclear protein localization protein 4 homolog [KO:K14015 ]
116085701 Ufd1; ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016 ]
116103294 Hspa6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283 ]
116074758 Dnajb12; dnaJ homolog subfamily B member 12 isoform X1 [KO:K09518 ]
116096033 Rad23b; UV excision repair protein RAD23 homolog B isoform X1 [KO:K10839 ]
116069726 Rad23a; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839 ]
116080060 Eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
116090338 Nfe2l2; nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638 ]
116076129 Atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
116102205 Ppp1r15a; protein phosphatase 1 regulatory subunit 15A [KO:K14019 ]
116103242 Atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054 ]
116075007 Atf6b; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049 ]
116076830 Ern1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852 ] [EC:2.7.11.1 3.1.26.-]
116069899 Herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X1 [KO:K14027 ]
Compound
Reference
Authors
Naidoo N
Title
ER and aging-Protein folding and the ER stress response.
Journal
Reference
Authors
Malhotra JD, Kaufman RJ
Title
The endoplasmic reticulum and the unfolded protein response.
Journal
Reference
Authors
Maattanen P, Gehring K, Bergeron JJ, Thomas DY
Title
Protein quality control in the ER: the recognition of misfolded proteins.
Journal
Reference
Authors
Stolz A, Wolf DH
Title
Endoplasmic reticulum associated protein degradation: a chaperone assisted journey to hell.
Journal
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
Related pathway
KO pathway
LinkDB
All DBs