Entry
Name
Protein processing in endoplasmic reticulum - Lynx rufus (bobcat)
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
lruf04141 Protein processing in endoplasmic reticulum
Ortholog table
Other DBs
Organism
Lynx rufus (bobcat) [GN:
lruf ]
Gene
124502268 protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956 ]
124524050 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666 ]
124503548 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 [KO:K12667 ]
124508801 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668 ]
124514765 dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit [KO:K12670 ]
124515315 dnaJ homolog subfamily C member 10 isoform X1 [KO:K09530 ] [EC:1.8.4.-]
124522323 endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like isoform X1 [KO:K23741 ] [EC:3.2.1.209 ]
124522354 endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like isoform X1 [KO:K23741 ] [EC:3.2.1.209 ]
124503305 prolactin regulatory element-binding protein isoform X1 [KO:K14003 ]
124513281 UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718 ] [EC:2.4.1.-]
124514701 UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
124523085 ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084 ]
124503048 ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085 ]
124510157 ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
124525856 translocon-associated protein subunit delta isoform X1 [KO:K04571 ]
124520546 translocating chain-associated membrane protein 1-like 1 [KO:K14010 ]
124525663 ubiquitin recognition factor in ER-associated degradation protein 1 isoform X1 [KO:K14016 ]
124526648 LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein-like [KO:K03283 ]
124520272 LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein-like [KO:K03283 ]
124527622 LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein-like [KO:K03283 ]
124503224 LOW QUALITY PROTEIN: dnaJ homolog subfamily C member 5G-like [KO:K09525 ]
124524077 UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839 ]
124514555 nuclear factor erythroid 2-related factor 2 isoform X1 [KO:K05638 ]
124509907 cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054 ]
124528650 cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049 ]
124518371 LOW QUALITY PROTEIN: uncharacterized protein LOC124518371 [KO:K09027 ]
124511331 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
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