PATHWAY: xla04141 Help
Entry
Name
Protein processing in endoplasmic reticulum - Xenopus laevis (African clawed frog)
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
xla04141 Protein processing in endoplasmic reticulum
Other DBs
Organism
Xenopus laevis (African clawed frog) [GN:
xla ]
Gene
100049149 edem1.L; ER degradation enhancing alpha-mannosidase like protein 1 L homeolog precursor [KO:K10084 ]
100137614 erlec1.L; endoplasmic reticulum lectin 1-like isoform X2 [KO:K14008 ]
100158335 plaa.L; phospholipase A2-activating protein L homeolog [KO:K14018 ]
100158355 hspbp1.L; HSPA (heat shock 70kDa) binding protein, cytoplasmic cochaperone 1 L homeolog [KO:K09562 ]
100174792 bag1.L; BAG family molecular chaperone regulator 1 isoform X1 [KO:K09555 ]
100380973 stt3b.S; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151 ] [EC:2.4.99.18 ]
100381020 hspa1a.S; heat shock protein family A (Hsp70) member 1A S homeolog [KO:K03283 ]
100381056 sec24a.S; protein transport protein Sec24A isoform X1 [KO:K14007 ]
108696249 sec24c.L; protein transport protein Sec24C isoform X1 [KO:K14007 ]
108696454 hyou1.L; hypoxia up-regulated protein 1-like isoform X1 [KO:K09486 ]
108696811 hspa1a.L; heat shock cognate 71 kDa protein-like isoform X1 [KO:K03283 ]
108696873 ddost.L; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit-like [KO:K12670 ]
108697581 cryab.S; LOW QUALITY PROTEIN: alpha-crystallin B chain [KO:K09542 ]
108698555 atf6b.L; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049 ]
108699293 eif2s1.L; eukaryotic translation initiation factor 2 subunit 1 isoform X2 [KO:K03237 ]
108700226 atf6b.S; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049 ]
108701068 edem2.L; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085 ]
108701211 ern1.L; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852 ] [EC:2.7.11.1 3.1.26.-]
108701402 dnajc10.L; dnaJ homolog subfamily C member 10-like [KO:K09530 ] [EC:1.8.4.-]
108702862 ern1.S; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852 ] [EC:2.7.11.1 3.1.26.-]
108704603 rpn2.L; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X1 [KO:K12667 ]
108706700 sec24b.S; protein transport protein Sec24B isoform X1 [KO:K14007 ]
108708482 uggt2.L; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718 ] [EC:2.4.1.-]
108710148 sec24b.L; protein transport protein Sec24B isoform X1 [KO:K14007 ]
108710987 lman2.L; vesicular integral-membrane protein VIP36 isoform X1 [KO:K10082 ]
108712619 skp1.S; S-phase kinase-associated protein 1 isoform X2 [KO:K03094 ]
108712658 map2k7.S; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431 ] [EC:2.7.12.2 ]
108714495 atf6.L; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X3 [KO:K09054 ]
108714662 sec61a1.L; protein transport protein Sec61 subunit alpha [KO:K10956 ]
108714902 dad1.L; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit dad1-like [KO:K12668 ]
108715710 edem3.S; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X2 [KO:K10086 ]
108715849 edem1.S; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084 ]
108716133 ero1b.L; ERO1-like protein beta isoform X1 [KO:K10976 ] [EC:1.8.4.-]
108717160 uggt1.L; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718 ] [EC:2.4.1.-]
108718938 stt3b.L; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151 ] [EC:2.4.99.18 ]
373637 bax.S; BCL2-associated X protein L homeolog [KO:K02159 ]
379209 nfe2l2.L; nuclear factor, erythroid 2 like 2 L homeolog [KO:K05638 ]
379319 hspa8.L; heat shock protein family A (Hsp70) member 8 L homeolog [KO:K03283 ]
379348 rpn2.S; ribophorin II S homeolog precursor [KO:K12667 ]
379368 ssr2.S; signal sequence receptor, beta precursor [KO:K13250 ]
379373 dnaja2.L; DnaJ heat shock protein family (Hsp40) member A2 L homeolog [KO:K09503 ]
379415 ssr3.S; signal sequence receptor gamma subunit b [KO:K13251 ]
379611 ssr1.L; signal sequence receptor, alpha (translocon-associated protein alpha) L homeolog precursor [KO:K13249 ]
379743 pdia3.S; protein disulfide isomerase family A member 3 S homeolog precursor [KO:K08056 ] [EC:5.3.4.1 ]
379756 hspa5.S; heat shock protein family A (Hsp70) member 5 S homeolog precursor [KO:K09490 ] [EC:3.6.4.10 ]
379760 hspa1l.S; heat shock protein family A (Hsp70) member 1 like S homeolog [KO:K03283 ]
379791 dnajc3.L; DnaJ heat shock protein family (Hsp40) member C3 L homeolog precursor [KO:K09523 ]
379848 sec61b.L; SEC61 translocon beta subunit L homeolog [KO:K09481 ]
379879 nsfl1c.L; NSFL1 (p97) cofactor (p47) L homeolog [KO:K14012 ]
379936 sec61a1.S; Sec61 translocon alpha 1 subunit S homeolog [KO:K10956 ]
379997 pdia6.S; protein disulfide isomerase family A member 6 S homeolog precursor [KO:K09584 ] [EC:5.3.4.1 ]
380008 ssr3.L; signal sequence receptor gamma subunit a [KO:K13251 ]
380057 dnaja1.L; DnaJ heat shock protein family (Hsp40) member A1 L homeolog [KO:K09502 ]
380119 nploc4.S; NPL4 homolog, ubiquitin recognition factor S homeolog [KO:K14015 ]
380134 sec13.S; SEC13 homolog, nuclear pore and COPII coat complex component S homeolog [KO:K14004 ]
380215 xbp1.L; X-box binding protein 1 L homeolog [KO:K09027 ]
380444 eif2s1.S; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
380491 vcp.S; transitional endoplasmic reticulum ATPase [KO:K13525 ]
380538 skp1.L; S-phase kinase-associated protein 1 [KO:K03094 ]
387598 sar1b.L; secretion associated, Ras related GTPase 1B L homeolog [KO:K07953 ] [EC:3.6.5.-]
394295 lman1.S; lectin, mannose binding 1 S homeolog precursor [KO:K10080 ]
394391 map2k7.L; dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431 ] [EC:2.7.12.2 ]
394410 herpud1.S; homocysteine inducible ER protein with ubiquitin like domain 1 S homeolog [KO:K14027 ]
394424 sec61g.L; protein transport protein Sec61 subunit gamma [KO:K07342 ]
397721 dad1.S; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit dad1 [KO:K12668 ]
397945 ssr4.L; signal sequence receptor, delta L homeolog precursor [KO:K04571 ]
398230 tram1.S; translocation associated membrane protein 1 S homeolog [KO:K14010 ]
398507 rad23b.S; RAD23 homolog B, nucleotide excision repair protein S homeolog [KO:K10839 ]
398514 rpn1.L; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 precursor [KO:K12666 ]
398521 rpn1.S; ribophorin I S homeolog precursor [KO:K12666 ]
398531 hyou1.S; hypoxia up-regulated protein 1 isoform X1 [KO:K09486 ]
398615 ddit3.L; DNA damage inducible transcript 3 L homeolog [KO:K04452 ]
398629 herpud1.L; homocysteine inducible ER protein with ubiquitin like domain 1 L homeolog [KO:K14027 ]
398753 hsp90b1.S; uncharacterized protein LOC398753 precursor [KO:K09487 ]
398804 atf4.L; activating transcription factor 4 L homeolog [KO:K04374 ]
399034 ufd1.S; ubiquitin fusion degradation protein 1 homolog [KO:K14016 ]
399123 dnajc5.S; dnaJ homolog subfamily C member 5 [KO:K09525 ]
399159 ufd1.L; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016 ]
399231 ssr1.S; signal sequence receptor, alpha (translocon-associated protein alpha) S homeolog precursor [KO:K13249 ]
399234 stt3a.L; STT3A, subunit of the oligosaccharyltransferase complex (catalytic) L homeolog [KO:K07151 ] [EC:2.4.99.18 ]
399408 hsp90b1.L; heat shock protein 90kDa beta family member 1 L homeolog precursor [KO:K09487 ]
403386 eif2ak3.S; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860 ] [EC:2.7.11.1 ]
414657 sil1.L; nucleotide exchange factor SIL1 precursor [KO:K14001 ]
431836 XB22063322.L; uncharacterized protein LOC431836 precursor [KO:K09523 ]
431990 dnajc10.S; dnaJ homolog subfamily C member 10 precursor [KO:K09530 ] [EC:1.8.4.-]
432145 dnajb2.L; DnaJ heat shock protein family (Hsp40) member B2 L homeolog [KO:K09508 ]
432348 sec31b.S; SEC31 homolog B, COPII coat complex component S homeolog [KO:K14005 ]
443698 plaa.S; phospholipase A-2-activating protein isoform X1 [KO:K14018 ]
443814 ubxn1.S; UBX domain-containing protein 1-A [KO:K24348 ]
443902 sec63.S; SEC63 homolog, protein translocation regulator S homeolog [KO:K09540 ]
443907 edem3.L; ER degradation-enhancing alpha-mannosidase-like protein 3 precursor [KO:K10086 ]
444075 ern2.S; endoplasmic reticulum to nucleus signaling 2 S homeolog precursor [KO:K08852 ] [EC:2.7.11.1 3.1.26.-]
444085 atxn3.L; ataxin-3 isoform X1 [KO:K11863 ] [EC:3.4.22.-]
444229 ostc.L; oligosaccharyltransferase complex subunit ostc-A [KO:K26501 ]
444260 dnajc1.S; DnaJ heat shock protein family (Hsp40) member C1 S homeolog precursor [KO:K09521 ]
444283 ddost.S; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit precursor [KO:K12670 ]
444366 rrbp1.S; ribosome binding protein 1 S homeolog [KO:K14000 ]
444375 dnajb12.S; DnaJ heat shock protein family (Hsp40) member B12 S homeolog [KO:K09518 ]
444485 preb.L; prolactin regulatory element binding L homeolog [KO:K14003 ]
444614 prkcsh.S; protein kinase C substrate 80K-H S homeolog precursor [KO:K08288 ]
444653 ubxn1.L; UBX domain-containing protein 1-B [KO:K24348 ]
444697 uggt2.S; UDP-glucose glycoprotein glucosyltransferase 2 S homeolog [KO:K11718 ] [EC:2.4.1.-]
444736 nfe2l2.S; nuclear factor, erythroid 2 like 2 S homeolog [KO:K05638 ]
444772 dnajb11.S; DnaJ heat shock protein family (Hsp40) member B11 S homeolog [KO:K09517 ]
444834 sec23b.L; Sec23 homolog B, COPII coat complex component L homeolog [KO:K14006 ]
446223 ubxn4.S; UBX domain-containing protein 4 isoform X1 [KO:K24348 ]
446313 hspb6.L; heat shock protein family B (small) member 6 L homeolog [KO:K09542 ]
446331 bcap31.L; B cell receptor associated protein 31 L homeolog [KO:K14009 ]
446459 hsp90ab1.S; heat shock protein 90kDa alpha family class B member 1 S homeolog [KO:K04079 ]
446460 tram1.L; translocating chain-associated membrane protein 1-like 1 [KO:K14010 ]
446478 pdia6.L; protein disulfide isomerase family A member 6 L homeolog precursor [KO:K09584 ] [EC:5.3.4.1 ]
446589 ubxn2a.L; UBX domain protein 2A L homeolog [KO:K24349 ]
446660 sec62.L; SEC62 homolog, preprotein translocation factor L homeolog [KO:K12275 ]
446676 sec62.S; SEC62 homolog, preprotein translocation factor S homeolog [KO:K12275 ]
446945 sec24d.L; protein transport protein Sec24D [KO:K14007 ]
447013 prkcsh.L; protein kinase C substrate 80K-H L homeolog precursor [KO:K08288 ]
447205 mbtps1.L; membrane-bound transcription factor site-1 protease preproprotein [KO:K08653 ] [EC:3.4.21.112 ]
447223 dnajc5g.L; DnaJ heat shock protein family (Hsp40) member C5 gamma L homeolog [KO:K09525 ]
447291 man1b1.L; mannosidase, alpha, class 1B, member 1 L homeolog [KO:K23741 ]
447425 sec31b.L; SEC31 homolog B, COPII coat complex component L homeolog [KO:K14005 ]
447508 sar1a.S; secretion associated, Ras related GTPase 1A S homeolog [KO:K07953 ] [EC:3.6.5.-]
447693 sec24c.S; SEC24 homolog C, COPII coat complex component S homeolog [KO:K14007 ]
494750 magt1.L; magnesium transporter protein 1 precursor [KO:K19478 ]
494816 nploc4.L; uncharacterized protein LOC494816 [KO:K14015 ]
495169 pdia4.L; protein disulfide isomerase family A member 4 L homeolog precursor [KO:K09582 ] [EC:5.3.4.1 ]
495416 sec63.L; SEC63 homolog, protein translocation regulator L homeolog [KO:K09540 ]
495829 erlec1.S; endoplasmic reticulum lectin 1 precursor [KO:K14008 ]
496056 atf6.S; activating transcription factor 6 S homeolog [KO:K09054 ]
496140 ostc.S; oligosaccharyltransferase complex subunit ostc-B [KO:K26501 ]
734165 atf4.S; activating transcription factor 4 S homeolog [KO:K04374 ]
734416 ckap4.L; cytoskeleton-associated protein 4 L homeolog [KO:K13999 ]
734481 rad23b.L; uncharacterized protein LOC734481 [KO:K10839 ]
734526 cul1.L; uncharacterized protein LOC734526 [KO:K03347 ]
734644 bak1.S; BCL2 antagonist/killer 1 S homeolog [KO:K14021 ]
734911 wfs1.L; Wolfram syndrome 1 (wolframin) L homeolog [KO:K14020 ]
735099 sel1l.L; sSEL1L adaptor subunit of ERAD E3 ubiquitin ligase L homeolog precursor [KO:K14026 ]
779022 stt3a.S; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A isoform X1 [KO:K07151 ] [EC:2.4.99.18 ]
779038 sec23a.S; protein transport protein Sec23B [KO:K14006 ]
779300 yod1.L; ubiquitin thioesterase OTU1 [KO:K13719 ] [EC:3.1.2.-]
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