Entry
Name
Protein processing in endoplasmic reticulum - Xenopus tropicalis (tropical 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
xtr04141 Protein processing in endoplasmic reticulum
Other DBs
Organism
Xenopus tropicalis (tropical clawed frog) [GN:
xtr ]
Gene
100125094 sec63; translocation protein SEC63 homolog isoform X1 [KO:K09540 ]
100145194 man1b1; endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase [KO:K23741 ]
100145597 ddost; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit precursor [KO:K12670 ]
100145667 edem3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X2 [KO:K10086 ]
100216289 nploc4; nuclear protein localization protein 4 homolog [KO:K14015 ]
100380058 uggt1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X3 [KO:K11718 ] [EC:2.4.1.-]
100485441 hspa1b; LOW QUALITY PROTEIN: heat shock 70 kDa protein [KO:K03283 ]
100488855 atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X2 [KO:K09049 ]
100492802 sec61g; protein transport protein Sec61 subunit gamma [KO:K07342 ]
100493790 uggt2; UDP-glucose:glycoprotein glucosyltransferase 2 [KO:K11718 ] [EC:2.4.1.-]
100495934 stt3b; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B [KO:K07151 ] [EC:2.4.99.18 ]
100498199 atf6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054 ]
387599 sar1a; GTP-binding protein SAR1a [KO:K07953 ] [EC:3.6.5.-]
394579 bcap31; B-cell receptor-associated protein 31 [KO:K14009 ]
394787 dnajb11; dnaJ homolog subfamily B member 11 [KO:K09517 ]
394983 prkcsh; glucosidase 2 subunit beta precursor [KO:K08288 ]
395031 rpn1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 precursor [KO:K12666 ]
395034 edem1; ER degradation-enhancing alpha-mannosidase-like protein 1 [KO:K10084 ]
407861 stt3a; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151 ] [EC:2.4.99.18 ]
407868 tram1; translocating chain-associated membrane protein 1 [KO:K14010 ]
407898 herpud1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein [KO:K14027 ]
407912 preb; prolactin regulatory element-binding protein [KO:K14003 ]
407930 erlec1; endoplasmic reticulum lectin 1 precursor [KO:K14008 ]
448005 atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
448030 ssr2; translocon-associated protein subunit beta precursor [KO:K13250 ]
448033 sec61a1; protein transport protein Sec61 subunit alpha isoform 1 [KO:K10956 ]
448048 dnaja2; dnaJ homolog subfamily A member 2 [KO:K09503 ]
448068 dnajc5g; DnaJ heat shock protein family (Hsp40) member C5 gamma [KO:K09525 ]
448087 eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
448177 vcp; transitional endoplasmic reticulum ATPase [KO:K13525 ]
448388 bag2; BAG family molecular chaperone regulator 2 [KO:K09556 ]
448516 ufd1; ubiquitin fusion degradation protein 1 homolog [KO:K14016 ]
448548 edem2; ER degradation-enhancing alpha-mannosidase-like protein 2 precursor [KO:K10085 ]
448655 ssr3; translocon-associated protein subunit gamma [KO:K13251 ]
493217 nfe2l2; nuclear factor erythroid 2-related factor 2 [KO:K05638 ]
493474 dnajb1; dnaJ homolog subfamily B member 1 [KO:K09507 ]
496421 dnaja1; dnaJ homolog subfamily A member 1 [KO:K09502 ]
496816 dnajc1; dnaJ homolog subfamily C member 1 precursor [KO:K09521 ]
496959 rpn2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 precursor [KO:K12667 ]
496977 dnajc3; dnaJ homolog subfamily C member 3 precursor [KO:K09523 ]
548454 dnajb2; dnaJ homolog subfamily B member 2 [KO:K09508 ]
548588 sar1b; GTP-binding protein SAR1b [KO:K07953 ] [EC:3.6.5.-]
548742 dnajc5; uncharacterized protein LOC548742 [KO:K09525 ]
548745 dnajb12; dnaJ homolog subfamily B member 12 [KO:K09518 ]
548812 ero1a; ERO1-like protein alpha precursor [KO:K10950 ] [EC:1.8.4.-]
549036 hsp90aa1.1; heat shock protein 90kDa alpha family class A member 1 [KO:K04079 ]
549273 skp1; S-phase kinase-associated protein 1 [KO:K03094 ]
549410 ostc; oligosaccharyltransferase complex subunit ostc [KO:K26501 ]
549666 ssr1; translocon-associated protein subunit alpha precursor [KO:K13249 ]
549679 dnajc10; dnaJ homolog subfamily C member 10 [KO:K09530 ] [EC:1.8.4.-]
549694 dad1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 [KO:K12668 ]
594966 rad23b; UV excision repair protein RAD23 homolog B [KO:K10839 ]
733816 ddit3; DNA damage-inducible transcript 3 protein [KO:K04452 ]
779444 hyou1; hypoxia up-regulated protein 1 isoform 1 precursor [KO:K09486 ]
779642 lman2; vesicular integral-membrane protein VIP36 precursor [KO:K10082 ]
779732 ern2; serine/threonine-protein kinase/endoribonuclease IRE2 precursor [KO:K08852 ] [EC:2.7.11.1 3.1.26.-]
779883 hspa1l; heat shock protein family A (Hsp70) member 1 like [KO:K03283 ]
780222 hsp90aa1.2; heat shock protein 90kDa alpha (cytosolic), class A member 1, gene 2 [KO:K04079 ]
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