KEGG   PATHWAY: cfa04141
Entry
cfa04141                    Pathway                                
Name
Protein processing in endoplasmic reticulum - Canis lupus familiaris (dog)
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
Pathway map
cfa04141  Protein processing in endoplasmic reticulum
cfa04141

Other DBs
GO: 0030433 0034976
Organism
Canis lupus familiaris (dog) [GN:cfa]
Gene
404006  SEC61A1; protein transport protein Sec61 subunit alpha isoform 1 [KO:K10956]
478007  SEC61A2; protein transport protein Sec61 subunit alpha isoform X1 [KO:K10956]
404018  SEC61B; protein transport protein Sec61 subunit beta [KO:K09481]
404017  SEC61G; protein transport protein Sec61 subunit gamma [KO:K07342]
106559577  protein transport protein Sec61 subunit gamma-like [KO:K07342]
609416  SEC62; translocation protein SEC62 [KO:K12275]
475016  SEC63; translocation protein SEC63 homolog [KO:K09540]
476516  RPN1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 [KO:K12666]
477223  RPN2; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform X3 [KO:K12667]
480238  DAD1; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 isoform X1 [KO:K12668]
404012  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit precursor [KO:K12670]
489300  STT3A; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A [KO:K07151] [EC:2.4.99.18]
485628  STT3B; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B isoform X1 [KO:K07151] [EC:2.4.99.18]
491975  MAGT1; magnesium transporter protein 1 isoform X2 [KO:K19478]
475608  TUSC3; tumor suppressor candidate 3 isoform X1 [KO:K19478]
100688081  oligosaccharyltransferase complex subunit OSTC [KO:K26501]
100684247  oligosaccharyltransferase complex subunit OSTC-like [KO:K26501]
477512  OSTC; oligosaccharyltransferase complex subunit OSTC [KO:K26501]
476064  TMEM258; transmembrane protein 258 isoform X2 [KO:K26502]
102153475  transmembrane protein 258-like isoform X3 [KO:K26502]
119872320  transmembrane protein 258-like [KO:K26502]
483104  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
481295  CKAP4; cytoskeleton-associated protein 4 [KO:K13999]
403809  RRBP1; ribosome-binding protein 1 isoform X1 [KO:K14000]
474699  SIL1; nucleotide exchange factor SIL1 isoform X1 [KO:K14001]
479410  HYOU1; hypoxia up-regulated protein 1 isoform X2 [KO:K09486]
480726  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
478664  DNAJB11; dnaJ homolog subfamily B member 11 isoform X3 [KO:K09517]
607587  DNAJC1; dnaJ homolog subfamily C member 1 [KO:K09521]
476966  DNAJC3; dnaJ homolog subfamily C member 3 [KO:K09523]
478826  DNAJC10; dnaJ homolog subfamily C member 10 [KO:K09530] [EC:1.8.4.-]
404019  HSP90B1; endoplasmin precursor [KO:K09487]
483784  GANAB; neutral alpha-glucosidase AB isoform X3 [KO:K05546] [EC:3.2.1.207]
484941  PRKCSH; glucosidase 2 subunit beta isoform X1 [KO:K08288]
403908  CANX; calnexin precursor [KO:K08054]
478279  PDIA3; protein disulfide-isomerase A3 [KO:K08056] [EC:5.3.4.1]
476694  CALR; calreticulin [KO:K08057]
483141  MAN1A2; mannosyl-oligosaccharide 1,2-alpha-mannosidase IB isoform X3 [KO:K01230] [EC:3.2.1.113]
476275  MAN1A1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IA [KO:K01230] [EC:3.2.1.113]
478178  MAN1C1; mannosyl-oligosaccharide 1,2-alpha-mannosidase IC isoform X1 [KO:K01230] [EC:3.2.1.113]
480667  endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase isoform X1 [KO:K23741] [EC:3.2.1.209]
403938  LMAN2; vesicular integral-membrane protein VIP36 precursor [KO:K10082]
476186  LMAN1; protein ERGIC-53 isoform X2 [KO:K10080]
487650  LMAN1L; protein ERGIC-53-like isoform X1 [KO:K10081]
483007  PREB; prolactin regulatory element-binding protein [KO:K14003]
479237  SAR1A; GTP-binding protein SAR1a isoform X1 [KO:K07953] [EC:3.6.5.-]
481509  SAR1B; GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
119876190  GTP-binding protein SAR1b [KO:K07953] [EC:3.6.5.-]
607653  SEC13; protein SEC13 homolog isoform X2 [KO:K14004]
478453  SEC31A; protein transport protein Sec31A isoform X1 [KO:K14005]
486842  SEC31B; protein transport protein Sec31B isoform X1 [KO:K14005]
477142  SEC23B; protein transport protein Sec23B isoform X1 [KO:K14006]
480295  SEC23A; protein transport protein Sec23A isoform X9 [KO:K14006]
487898  SEC24B; protein transport protein Sec24B isoform X2 [KO:K14007]
481510  SEC24A; protein transport protein Sec24A [KO:K14007]
478523  SEC24D; protein transport protein Sec24D isoform X1 [KO:K14007]
100855957  SEC24C; protein transport protein Sec24C isoform X2 [KO:K14007]
485525  UGGT2; UDP-glucose:glycoprotein glucosyltransferase 2 isoform X2 [KO:K11718] [EC:2.4.1.-]
476101  UGGT1; UDP-glucose:glycoprotein glucosyltransferase 1 isoform X1 [KO:K11718] [EC:2.4.1.-]
476547  EDEM1; LOW QUALITY PROTEIN: ER degradation-enhancing alpha-mannosidase-like protein 1 isoform X1 [KO:K10084]
477209  EDEM2; ER degradation-enhancing alpha-mannosidase-like protein 2 [KO:K10085]
480040  EDEM3; ER degradation-enhancing alpha-mannosidase-like protein 3 isoform X2 [KO:K10086]
483369  P4HB; protein disulfide-isomerase [KO:K09580] [EC:5.3.4.1]
482715  PDIA4; protein disulfide-isomerase A4 [KO:K09582] [EC:5.3.4.1]
475668  PDIA6; protein disulfide-isomerase A6 [KO:K09584] [EC:5.3.4.1]
477482  ERP29; endoplasmic reticulum resident protein 29 [KO:K09586]
100683583  TXNDC5; LOW QUALITY PROTEIN: thioredoxin domain-containing protein 5 isoform X1 [KO:K13984] [EC:5.3.4.1]
490691  ERO1A; ERO1-like protein alpha [KO:K10950] [EC:1.8.4.-]
488956  ERO1B; ERO1-like protein beta isoform X2 [KO:K10976] [EC:1.8.4.-]
474419  OS9; protein OS-9 isoform X1 [KO:K10088]
474593  ERLEC1; endoplasmic reticulum lectin 1 isoform X2 [KO:K14008]
403951  SSR1; translocon-associated protein subunit alpha precursor [KO:K13249]
403950  SSR2; translocon-associated protein subunit beta precursor [KO:K13250]
477124  SSR3; translocon-associated protein subunit gamma [KO:K13251]
481082  SSR4; translocon-associated protein subunit delta precursor [KO:K04571]
481080  BCAP31; B-cell receptor-associated protein 31 isoform X2 [KO:K14009]
403948  TRAM1; translocating chain-associated membrane protein 1 [KO:K14010]
487917  TRAM1L1; translocating chain-associated membrane protein 1-like 1 [KO:K14010]
475086  DERL1; derlin-1 isoform X1 [KO:K11519]
606991  DERL2; derlin-2 isoform X1 [KO:K13989]
486406  DERL3; derlin-3 isoform X1 [KO:K13989]
476130  UBXN4; UBX domain-containing protein 4 [KO:K24348]
476055  UBXN1; UBX domain-containing protein 1 [KO:K24348]
482992  UBXN2A; UBX domain-containing protein 2A isoform X1 [KO:K24349]
485037  UBXN6; UBX domain-containing protein 6 isoform X3 [KO:K14011]
475595  UBXN8; UBX domain-containing protein 8 isoform X2 [KO:K24351]
100685961  NSFL1C; NSFL1 cofactor p47 isoform X1 [KO:K14012]
100500800  SVIP; small VCP/p97-interacting protein [KO:K14014]
481590  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
483363  NPLOC4; nuclear protein localization protein 4 homolog isoform X1 [KO:K14015]
608300  UFD1; ubiquitin recognition factor in ER-associated degradation protein 1 [KO:K14016]
474850  heat shock 70 kDa protein 1-like isoform X1 [KO:K03283]
403612  HSP70; heat shock 70 kDa protein 1 [KO:K03283]
479406  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
119873859  LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein-like isoform X2 [KO:K03283]
607182  heat shock cognate 71 kDa protein-like [KO:K03283]
119863874  dnaJ homolog subfamily A member 1 [KO:K09502]
478143  DNAJA2; dnaJ homolog subfamily A member 2 [KO:K09503]
476688  DNAJB1; dnaJ homolog subfamily B member 1 isoform X2 [KO:K09507]
100683427  DNAJB2; dnaJ homolog subfamily B member 2 isoform X1 [KO:K09508]
489039  DNAJB12; dnaJ homolog subfamily B member 12 isoform X2 [KO:K09518]
610015  DNAJC5B; dnaJ homolog subfamily C member 5B isoform X6 [KO:K09525]
611034  DNAJC5G; dnaJ homolog subfamily C member 5G isoform X2 [KO:K09525]
480438  HSP90AA1; heat shock protein HSP 90-alpha [KO:K04079]
474919  HSP90AB1; heat shock protein HSP 90-beta [KO:K04079]
100687242  LOW QUALITY PROTEIN: heat shock protein HSP 90-beta-like [KO:K04079]
476089  HSPA4L; heat shock 70 kDa protein 4L isoform X2 [KO:K09485]
477322  HSPH1; heat shock protein 105 kDa isoform X1 [KO:K09485]
611781  BAG1; BAG family molecular chaperone regulator 1 isoform X2 [KO:K09555]
610506  BAG2; BAG family molecular chaperone regulator 2 [KO:K09556]
476381  HSPBP1; hsp70-binding protein 1 [KO:K09562]
487786  CRYAA; alpha-crystallin A chain [KO:K09541]
479441  CRYAB; alpha-crystallin B chain [KO:K09542]
490268  YOD1; ubiquitin thioesterase OTU1 isoform X2 [KO:K13719] [EC:3.1.2.-]
474733  PLAA; phospholipase A-2-activating protein isoform X2 [KO:K14018]
481656  RAD23B; UV excision repair protein RAD23 homolog B isoform X2 [KO:K10839]
484922  RAD23A; UV excision repair protein RAD23 homolog A isoform X1 [KO:K10839]
476312  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
476826  UBQLN3; ubiquilin-3 [KO:K04523]
490419  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
491909  UBQLN2; ubiquilin-2 [KO:K04523]
100682555  NGLY1; peptide-N(4)-(N-acetyl-beta- glucosaminyl)asparagine amidase isoform X1 [KO:K01456] [EC:3.5.1.52]
480229  ATXN3; LOW QUALITY PROTEIN: ataxin-3 [KO:K11863] [EC:3.4.22.-]
100687064  LOW QUALITY PROTEIN: ataxin-3 isoform X6 [KO:K11863] [EC:3.4.22.-]
479603  UBE4B; ubiquitin conjugation factor E4 B isoform X1 [KO:K10597] [EC:2.3.2.27]
475756  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X2 [KO:K08860] [EC:2.7.11.1]
480361  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
478813  NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X3 [KO:K05638]
474503  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
476419  PPP1R15A; protein phosphatase 1 regulatory subunit 15A [KO:K14019]
607439  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
403416  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
488660  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
474855  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
482113  WFS1; wolframin isoform X1 [KO:K14020]
489684  MBTPS1; membrane-bound transcription factor site-1 protease isoform X1 [KO:K08653] [EC:3.4.21.112]
477532  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
610766  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X6 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
480673  TRAF2; TNF receptor-associated factor 2 isoform X2 [KO:K03173] [EC:2.3.2.27]
476215  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
485004  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
477746  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
478464  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
474652  MAPK9; mitogen-activated protein kinase 9 isoform X4 [KO:K04440] [EC:2.7.11.24]
403523  BAX; apoptosis regulator BAX [KO:K02159]
481744  BAK1; bcl-2 homologous antagonist/killer [KO:K14021]
483745  CAPN1; calpain-1 catalytic subunit [KO:K01367] [EC:3.4.22.52]
480118  CAPN2; calpain-2 catalytic subunit [KO:K03853] [EC:3.4.22.53]
479458  CASP12; caspase-12 [KO:K04741] [EC:3.4.22.-]
478617  MARCHF6; E3 ubiquitin-protein ligase MARCHF6 isoform X2 [KO:K10661] [EC:2.3.2.27]
474993  UBE2J1; ubiquitin-conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
606935  UBE2J2; ubiquitin-conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
100685242  UBE2G1; ubiquitin-conjugating enzyme E2 G1 isoform X1 [KO:K10575] [EC:2.3.2.23]
611581  UBE2G2; ubiquitin-conjugating enzyme E2 G2 isoform X3 [KO:K04555] [EC:2.3.2.23]
483746  SYVN1; E3 ubiquitin-protein ligase synoviolin isoform X2 [KO:K10601] [EC:2.3.2.27]
474858  RNF5; E3 ubiquitin-protein ligase RNF5 isoform X8 [KO:K10666] [EC:2.3.2.27]
486362  RNF185; E3 ubiquitin-protein ligase RNF185 isoform X3 [KO:K10666] [EC:2.3.2.27]
607791  SELENOS; selenoprotein S [KO:K14025]
480409  SEL1L; protein sel-1 homolog 1 isoform X1 [KO:K14026]
485763  SEL1L2; protein sel-1 homolog 2 isoform X17 [KO:K14026]
478116  HERPUD1; homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein isoform X3 [KO:K14027]
487269  AMFR; E3 ubiquitin-protein ligase AMFR isoform X3 [KO:K10636] [EC:2.3.2.36]
479892  STUB1; E3 ubiquitin-protein ligase CHIP [KO:K09561] [EC:2.3.2.27]
478495  UBE2D3; ubiquitin-conjugating enzyme E2 D3 isoform X2 [KO:K06689] [EC:2.3.2.23]
608578  UBE2D1; ubiquitin-conjugating enzyme E2 D1 [KO:K06689] [EC:2.3.2.23]
612742  UBE2D2; ubiquitin-conjugating enzyme E2 D2 isoform X1 [KO:K06689] [EC:2.3.2.23]
612316  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
474494  RBX1; E3 ubiquitin-protein ligase RBX1 isoform X2 [KO:K03868] [EC:2.3.2.32]
475512  CUL1; cullin-1 [KO:K03347]
474682  SKP1; S-phase kinase-associated protein 1 [KO:K03094]
111090648  S-phase kinase-associated protein 1-like [KO:K03094]
478231  FBXO2; F-box only protein 2 isoform X1 [KO:K10099]
487444  FBXO6; F-box only protein 6 [KO:K10100]
Compound
C00076  Calcium cation
G00009   
G00010   
G00011   
G00012   
G10694   
Reference
PMID:19491040
  Authors
Naidoo N
  Title
ER and aging-Protein folding and the ER stress response.
  Journal
Ageing Res Rev 8:150-9 (2009)
DOI:10.1016/j.arr.2009.03.001
Reference
PMID:18023214
  Authors
Malhotra JD, Kaufman RJ
  Title
The endoplasmic reticulum and the unfolded protein response.
  Journal
Semin Cell Dev Biol 18:716-31 (2007)
DOI:10.1016/j.semcdb.2007.09.003
Reference
PMID:20347046
  Authors
Maattanen P, Gehring K, Bergeron JJ, Thomas DY
  Title
Protein quality control in the ER: the recognition of misfolded proteins.
  Journal
Semin Cell Dev Biol 21:500-11 (2010)
DOI:10.1016/j.semcdb.2010.03.006
Reference
PMID:20219571
  Authors
Stolz A, Wolf DH
  Title
Endoplasmic reticulum associated protein degradation: a chaperone assisted journey to hell.
  Journal
Biochim Biophys Acta 1803:694-705 (2010)
DOI:10.1016/j.bbamcr.2010.02.005
Reference
PMID:20112977
  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
J Proteome Res 9:1763-71 (2010)
DOI:10.1021/pr900900x
Related
pathway
cfa00510  N-Glycan biosynthesis
cfa03050  Proteasome
cfa03060  Protein export
cfa04210  Apoptosis
cfa04930  Type II diabetes mellitus
cfa05010  Alzheimer disease
cfa05012  Parkinson disease
cfa05020  Prion disease
KO pathway
ko04141   
LinkDB

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