Entry
Name
Protein processing in endoplasmic reticulum - Scleropages formosus (Asian bonytongue)
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
sfm04141 Protein processing in endoplasmic reticulum
Ortholog table
Other DBs
Organism
Scleropages formosus (Asian bonytongue) [GN:
sfm ]
Gene
108935440 sec61g; protein transport protein Sec61 subunit gamma [KO:K07342 ]
108921095 sec63; translocation protein SEC63 homolog isoform X1 [KO:K09540 ]
108923400 rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666 ]
108923427 rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667 ]
108937910 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit dad1-like [KO:K12668 ]
108929574 dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668 ]
108933892 ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670 ]
108932108 stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151 ] [EC:2.4.99.18 ]
108942511 stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151 ] [EC:2.4.99.18 ]
108920220 dnajc10; dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530 ] [EC:1.8.4.-]
108938629 man1b1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741 ] [EC:3.2.1.209 ]
108922819 lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082 ]
108937137 preb; prolactin regulatory element-binding protein isoform X1 [KO:K14003 ]
108939728 sar1a; GTP-binding protein SAR1a isoform X1 [KO:K07953 ] [EC:3.6.5.-]
108936715 uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
108928533 uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
108937030 edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084 ]
108936922 ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085 ]
108919794 ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
108922651 ER degradation-enhancing alpha-mannosidase-like protein 3 [KO:K10086 ]
108919637 ero1a; ERO1-like protein alpha isoform X1 [KO:K10950 ] [EC:1.8.4.-]
108933755 ero1b; ERO1-like protein beta isoform X1 [KO:K10976 ] [EC:1.8.4.-]
108928011 ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249 ]
108936976 translocating chain-associated membrane protein 1-like [KO:K14010 ]
108935532 tram1; translocating chain-associated membrane protein 1 [KO:K14010 ]
108930289 nploc4; nuclear protein localization protein 4 homolog [KO:K14015 ]
108920131 ufd1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016 ]
108925188 dnajc5g; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525 ]
108929619 dnajc5b; dnaJ homolog subfamily C member 5B isoform X1 [KO:K09525 ]
108938797 plaa; phospholipase A-2-activating protein isoform X1 [KO:K14018 ]
108939805 rad23a; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839 ]
108919930 UV excision repair protein RAD23 homolog A-like isoform X1 [KO:K10839 ]
108923993 eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
108930667 atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
108935502 cyclic AMP-dependent transcription factor ATF-6 alpha-like [KO:K09054 ]
108935180 cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054 ]
108935684 atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049 ]
108925673 LOW QUALITY PROTEIN: uncharacterized protein LOC108925673 [KO:K09027 ]
108927413 ern2; serine/threonine-protein kinase/endoribonuclease IRE2 isoform X1 [KO:K08852 ] [EC:2.7.11.1 3.1.26.-]
108918942 herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [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
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