Entry
Name
Protein processing in endoplasmic reticulum - Poecilia latipinna (sailfin molly)
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
plai04141 Protein processing in endoplasmic reticulum
Ortholog table
Other DBs
Organism
Poecilia latipinna (sailfin molly) [GN:
plai ]
Gene
106958414 protein transport protein Sec61 subunit alpha-like isoform X1 [KO:K10956 ]
106950782 sec61g; protein transport protein Sec61 subunit gamma [KO:K07342 ]
106953246 rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666 ]
106934427 rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667 ]
106938528 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2-like [KO:K12667 ]
106962977 dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668 ]
106961944 ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit isoform X1 [KO:K12670 ]
106951578 stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151 ] [EC:2.4.99.18 ]
106954228 stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151 ] [EC:2.4.99.18 ]
106938199 LOW QUALITY PROTEIN: nucleotide exchange factor SIL1-like [KO:K14001 ]
106938780 LOW QUALITY PROTEIN: nucleotide exchange factor SIL1-like [KO:K14001 ]
106946411 dnajc10; dnaJ homolog subfamily C member 10 [KO:K09530 ] [EC:1.8.4.-]
106936359 endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase-like isoform X1 [KO:K23741 ] [EC:3.2.1.209 ]
106949924 lman2; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082 ]
106955006 uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
106957499 uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
106949451 edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084 ]
106943133 edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 isoform X1 [KO:K10085 ]
106948181 ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
106961222 edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X1 [KO:K10086 ]
106934485 ssr1; translocon-associated protein subunit alpha isoform X1 [KO:K13249 ]
106943373 bcap31; B-cell receptor-associated protein 31 isoform X1 [KO:K14009 ]
106961315 translocating chain-associated membrane protein 1-like 1 [KO:K14010 ]
106937677 tram1; translocating chain-associated membrane protein 1 [KO:K14010 ]
106945994 transitional endoplasmic reticulum ATPase-like isoform X1 [KO:K13525 ]
106960896 nploc4; nuclear protein localization protein 4 homolog [KO:K14015 ]
106956801 ufd1l; ubiquitin fusion degradation protein 1 homolog [KO:K14016 ]
106945881 LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein-like [KO:K03283 ]
106949233 dnajc5g; dnaJ homolog subfamily C member 5G isoform X1 [KO:K09525 ]
106945064 plaa; phospholipase A-2-activating protein isoform X1 [KO:K14018 ]
106948451 rad23a; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839 ]
106957937 eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
106963917 atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054 ]
106958253 atf6b; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049 ]
106963411 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
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