Prion disease Gene Set

Dataset KEGG Pathways 2026
Category structural or functional annotations
Type pathway
Description A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA; ATAXIA; and a fatal outcome. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The older literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83) (Experimental Factor Ontology, EFO_0004720)
External Link https://www.kegg.jp/entry/hsa05020
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Genes

268 proteins participating in the Prion disease pathway from the KEGG Pathways 2026 dataset.

Symbol Name
ADRM1 adhesion regulating molecule 1
AKR1C1 aldo-keto reductase family 1, member C1
APAF1 apoptotic peptidase activating factor 1
ATF4 activating transcription factor 4
ATF6B activating transcription factor 6 beta
ATP5F1A ATP synthase F1 subunit alpha
ATP5F1B ATP synthase F1 subunit beta
ATP5F1C ATP synthase F1 subunit gamma
ATP5F1D ATP synthase F1 subunit delta
ATP5F1E ATP synthase F1 subunit epsilon
ATP5MC1 ATP synthase membrane subunit c locus 1
ATP5MC2 ATP synthase membrane subunit c locus 2
ATP5MC3 ATP synthase membrane subunit c locus 3
ATP5PB ATP synthase peripheral stalk-membrane subunit b
ATP5PD ATP synthase peripheral stalk subunit d
ATP5PF ATP synthase peripheral stalk subunit F6
ATP5PO ATP synthase peripheral stalk subunit OSCP
ATP6 ATP synthase F0 subunit 6
ATP8 ATP synthase F0 subunit 8
BAD BCL2-associated agonist of cell death
BAX BCL2-associated X protein
C1QA complement component 1, q subcomponent, A chain
C1QB complement component 1, q subcomponent, B chain
C1QC complement component 1, q subcomponent, C chain
C5 complement component 5
C8A complement component 8, alpha polypeptide
C8B complement component 8, beta polypeptide
C8G complement component 8, gamma polypeptide
CACNA1B calcium channel, voltage-dependent, N type, alpha 1B subunit
CACNA1C calcium channel, voltage-dependent, L type, alpha 1C subunit
CACNA1D calcium channel, voltage-dependent, L type, alpha 1D subunit
CACNA1F calcium channel, voltage-dependent, L type, alpha 1F subunit
CACNA1S calcium channel, voltage-dependent, L type, alpha 1S subunit
CASP12 caspase 12 (gene/pseudogene)
CASP3 caspase 3, apoptosis-related cysteine peptidase
CASP9 caspase 9, apoptosis-related cysteine peptidase
CAV1 caveolin 1, caveolae protein, 22kDa
CAV2 caveolin 2
CAV3 caveolin 3
CCL5 chemokine (C-C motif) ligand 5
CCNI cyclin I
CLSTN1 calsyntenin 1
COX4I1 cytochrome c oxidase subunit IV isoform 1
COX4I2 cytochrome c oxidase subunit IV isoform 2 (lung)
COX5A cytochrome c oxidase subunit Va
COX5B cytochrome c oxidase subunit Vb
COX6A1 cytochrome c oxidase subunit VIa polypeptide 1
COX6A2 cytochrome c oxidase subunit VIa polypeptide 2
COX6B1 cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous)
COX6B2 cytochrome c oxidase subunit VIb polypeptide 2 (testis)
COX6C cytochrome c oxidase subunit VIc
COX7A1 cytochrome c oxidase subunit VIIa polypeptide 1 (muscle)
COX7A2 cytochrome c oxidase subunit VIIa polypeptide 2 (liver)
COX7A2L cytochrome c oxidase subunit VIIa polypeptide 2 like
COX7B cytochrome c oxidase subunit VIIb
COX7B2 cytochrome c oxidase subunit VIIb2
COX7C cytochrome c oxidase subunit VIIc
COX8A cytochrome c oxidase subunit VIIIA (ubiquitous)
COX8C cytochrome c oxidase subunit VIIIC
COXFA4 cytochrome c oxidase associated subunit FA4
COXFA4L2 cytochrome c oxidase hypoxia associated subunit FA4L2
CREB1 cAMP responsive element binding protein 1
CREB3L1 cAMP responsive element binding protein 3-like 1
CREB3L2 cAMP responsive element binding protein 3-like 2
CREB3L3 cAMP responsive element binding protein 3-like 3
CREB3L4 cAMP responsive element binding protein 3-like 4
CREB5 cAMP responsive element binding protein 5
CSNK2A2 casein kinase 2, alpha prime polypeptide
CSNK2A3 casein kinase 2, alpha 3 polypeptide
CSNK2B casein kinase 2, beta polypeptide
CXCL10 chemokine (C-X-C motif) ligand 10
CYBA cytochrome b-245, alpha polypeptide
CYBB cytochrome b-245, beta polypeptide
CYCS cytochrome c, somatic
CYTB cytochrome b
DDIT3 DNA-damage-inducible transcript 3
EGR1 early growth response 1
EIF2AK3 eukaryotic translation initiation factor 2-alpha kinase 3
EIF2S1 eukaryotic translation initiation factor 2, subunit 1 alpha, 35kDa
FYN FYN proto-oncogene, Src family tyrosine kinase
GDNF glial cell derived neurotrophic factor
GPRIN1 G protein regulated inducer of neurite outgrowth 1
GRIN2A glutamate receptor, ionotropic, N-methyl D-aspartate 2A
GRIN2B glutamate receptor, ionotropic, N-methyl D-aspartate 2B
GRIN2C glutamate receptor, ionotropic, N-methyl D-aspartate 2C
GRIN2D glutamate receptor, ionotropic, N-methyl D-aspartate 2D
GRIN3A glutamate receptor, ionotropic, N-methyl-D-aspartate 3A
GRIN3B glutamate receptor, ionotropic, N-methyl-D-aspartate 3B
GSK3B glycogen synthase kinase 3 beta
HSPA1A heat shock 70kDa protein 1A
HSPA1B heat shock 70kDa protein 1B
HSPA1L heat shock 70kDa protein 1-like
HSPA5 heat shock 70kDa protein 5 (glucose-regulated protein, 78kDa)
HSPA6 heat shock 70kDa protein 6 (HSP70B')
HSPA8 heat shock 70kDa protein 8
IL1A interleukin 1, alpha
IL1B interleukin 1, beta
IL6 interleukin 6
ITPR1 inositol 1,4,5-trisphosphate receptor, type 1
ITPR2 inositol 1,4,5-trisphosphate receptor, type 2
ITPR3 inositol 1,4,5-trisphosphate receptor, type 3
IVNS1ABP influenza virus NS1A binding protein
KIF5A kinesin family member 5A
KIF5B kinesin family member 5B
KIF5C kinesin family member 5C
KLC1 kinesin light chain 1
KLC3 kinesin light chain 3
KLC4 kinesin light chain 4
LAMC1 laminin, gamma 1 (formerly LAMB2)
LNCEGFL7OS lncRNA EGFL7 opposite strand
MAPK1 mitogen-activated protein kinase 1
MAPK10 mitogen-activated protein kinase 10
MAPK11 mitogen-activated protein kinase 11
MAPK12 mitogen-activated protein kinase 12
MAPK13 mitogen-activated protein kinase 13
MAPK14 mitogen-activated protein kinase 14
MAPK3 mitogen-activated protein kinase 3
MAPK8 mitogen-activated protein kinase 8
MAPK9 mitogen-activated protein kinase 9
MCU mitochondrial calcium uniporter
NCAM1 neural cell adhesion molecule 1
NCAM2 neural cell adhesion molecule 2
NCF1 neutrophil cytosolic factor 1
NCF2 neutrophil cytosolic factor 2
NCF4 neutrophil cytosolic factor 4, 40kDa
ND2 NADH dehydrogenase subunit 2
ND3 NADH dehydrogenase subunit 3
ND4 NADH dehydrogenase subunit 4
ND4L NADH dehydrogenase subunit 4L
ND5 NADH dehydrogenase subunit 5
ND6 NADH dehydrogenase subunit 6
NDUFA1 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa
NDUFA10 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa
NDUFA11 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 11, 14.7kDa
NDUFA12 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 12
NDUFA13 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13
NDUFA2 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2, 8kDa
NDUFA3 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 3, 9kDa
NDUFA5 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 5
NDUFA6 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6, 14kDa
NDUFA7 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 7, 14.5kDa
NDUFA8 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 8, 19kDa
NDUFA9 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9, 39kDa
NDUFAB1 NADH dehydrogenase (ubiquinone) 1, alpha/beta subcomplex, 1, 8kDa
NDUFB1 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 1, 7kDa
NDUFB10 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 10, 22kDa
NDUFB11 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11, 17.3kDa
NDUFB2 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 2, 8kDa
NDUFB3 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3, 12kDa
NDUFB4 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 4, 15kDa
NDUFB5 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5, 16kDa
NDUFB6 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6, 17kDa
NDUFB7 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 7, 18kDa
NDUFB8 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 8, 19kDa
NDUFB9 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 9, 22kDa
NDUFC1 NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 1, 6kDa
NDUFC2 NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2, 14.5kDa
NDUFC2-KCTD14 NDUFC2-KCTD14 readthrough
NDUFS1 NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase)
NDUFS2 NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase)
NDUFS3 NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase)
NDUFS4 NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase)
NDUFS5 NADH dehydrogenase (ubiquinone) Fe-S protein 5, 15kDa (NADH-coenzyme Q reductase)
NDUFS6 NADH dehydrogenase (ubiquinone) Fe-S protein 6, 13kDa (NADH-coenzyme Q reductase)
NDUFS7 NADH dehydrogenase (ubiquinone) Fe-S protein 7, 20kDa (NADH-coenzyme Q reductase)
NDUFS8 NADH dehydrogenase (ubiquinone) Fe-S protein 8, 23kDa (NADH-coenzyme Q reductase)
NDUFV1 NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa
NDUFV2 NADH dehydrogenase (ubiquinone) flavoprotein 2, 24kDa
NDUFV3 NADH dehydrogenase (ubiquinone) flavoprotein 3, 10kDa
NOTCH1 notch 1
PIK3CA phosphatidylinositol-4,5-bisphosphate 3-kinase, catalytic subunit alpha
PIK3CB phosphatidylinositol-4,5-bisphosphate 3-kinase, catalytic subunit beta
PIK3R1 phosphoinositide-3-kinase, regulatory subunit 1 (alpha)
PIK3R2 phosphoinositide-3-kinase, regulatory subunit 2 (beta)
PIK3R3 phosphoinositide-3-kinase, regulatory subunit 3 (gamma)
PPIF peptidylprolyl isomerase F
PPP3CA protein phosphatase 3, catalytic subunit, alpha isozyme
PPP3CB protein phosphatase 3, catalytic subunit, beta isozyme
PPP3CC protein phosphatase 3, catalytic subunit, gamma isozyme
PPP3R1 protein phosphatase 3, regulatory subunit B, alpha
PPP3R2 protein phosphatase 3, regulatory subunit B, beta
PRKACB protein kinase, cAMP-dependent, catalytic, beta
PRKACG protein kinase, cAMP-dependent, catalytic, gamma
PRKCA protein kinase C, alpha
PRKCD protein kinase C, delta
PRNP prion protein
PSMA1 proteasome (prosome, macropain) subunit, alpha type, 1
PSMA2 proteasome (prosome, macropain) subunit, alpha type, 2
PSMA3 proteasome (prosome, macropain) subunit, alpha type, 3
PSMA4 proteasome (prosome, macropain) subunit, alpha type, 4
PSMA5 proteasome (prosome, macropain) subunit, alpha type, 5
PSMA6 proteasome (prosome, macropain) subunit, alpha type, 6
PSMA7 proteasome (prosome, macropain) subunit, alpha type, 7
PSMA8 proteasome (prosome, macropain) subunit, alpha type, 8
PSMB1 proteasome (prosome, macropain) subunit, beta type, 1
PSMB2 proteasome (prosome, macropain) subunit, beta type, 2
PSMB3 proteasome (prosome, macropain) subunit, beta type, 3
PSMB4 proteasome (prosome, macropain) subunit, beta type, 4
PSMB5 proteasome (prosome, macropain) subunit, beta type, 5
PSMB6 proteasome (prosome, macropain) subunit, beta type, 6
PSMB7 proteasome (prosome, macropain) subunit, beta type, 7
PSMC1 proteasome (prosome, macropain) 26S subunit, ATPase, 1
PSMC2 proteasome (prosome, macropain) 26S subunit, ATPase, 2
PSMC3 proteasome (prosome, macropain) 26S subunit, ATPase, 3
PSMC4 proteasome (prosome, macropain) 26S subunit, ATPase, 4
PSMC5 proteasome (prosome, macropain) 26S subunit, ATPase, 5
PSMC6 proteasome (prosome, macropain) 26S subunit, ATPase, 6
PSMD1 proteasome (prosome, macropain) 26S subunit, non-ATPase, 1
PSMD11 proteasome (prosome, macropain) 26S subunit, non-ATPase, 11
PSMD12 proteasome (prosome, macropain) 26S subunit, non-ATPase, 12
PSMD13 proteasome (prosome, macropain) 26S subunit, non-ATPase, 13
PSMD14 proteasome (prosome, macropain) 26S subunit, non-ATPase, 14
PSMD2 proteasome (prosome, macropain) 26S subunit, non-ATPase, 2
PSMD3 proteasome (prosome, macropain) 26S subunit, non-ATPase, 3
PSMD4 proteasome (prosome, macropain) 26S subunit, non-ATPase, 4
PSMD6 proteasome (prosome, macropain) 26S subunit, non-ATPase, 6
PSMD7 proteasome (prosome, macropain) 26S subunit, non-ATPase, 7
PSMD8 proteasome (prosome, macropain) 26S subunit, non-ATPase, 8
PSMD9 proteasome (prosome, macropain) 26S subunit, non-ATPase, 9
PTGS1 prostaglandin-endoperoxide synthase 1 (prostaglandin G/H synthase and cyclooxygenase)
PTGS2 prostaglandin-endoperoxide synthase 2 (prostaglandin G/H synthase and cyclooxygenase)
RAC2 ras-related C3 botulinum toxin substrate 2 (rho family, small GTP binding protein Rac2)
RNASE1 ribonuclease, RNase A family, 1 (pancreatic)
RYR1 ryanodine receptor 1 (skeletal)
RYR2 ryanodine receptor 2 (cardiac)
RYR3 ryanodine receptor 3
SDHA succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
SDHB succinate dehydrogenase complex, subunit B, iron sulfur (Ip)
SDHC succinate dehydrogenase complex, subunit C, integral membrane protein, 15kDa
SDHD succinate dehydrogenase complex, subunit D, integral membrane protein
SEM1 SEM1 26S proteasome subunit
SLC25A31 solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 31
SLC25A4 solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 4
SLC25A5 solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 5
SLC25A6 solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 6
SOD1 superoxide dismutase 1, soluble
STIP1 stress-induced phosphoprotein 1
TNF tumor necrosis factor
TUBA1A tubulin, alpha 1a
TUBA1B tubulin, alpha 1b
TUBA1C tubulin, alpha 1c
TUBA3C tubulin, alpha 3c
TUBA3D tubulin, alpha 3d
TUBA3E tubulin, alpha 3e
TUBA4A tubulin, alpha 4a
TUBA8 tubulin, alpha 8
TUBAL3 tubulin, alpha-like 3
TUBB tubulin, beta class I
TUBB2A tubulin, beta 2A class IIa
TUBB2B tubulin, beta 2B class IIb
TUBB3 tubulin, beta 3 class III
TUBB4A tubulin, beta 4A class IVa
TUBB4B tubulin, beta 4B class IVb
TUBB6 tubulin, beta 6 class V
TUBB8 tubulin, beta 8 class VIII
TUBB8B tubulin beta 8B
UQCR10 ubiquinol-cytochrome c reductase, complex III subunit X
UQCR11 ubiquinol-cytochrome c reductase, complex III subunit XI
UQCRB ubiquinol-cytochrome c reductase binding protein
UQCRC1 ubiquinol-cytochrome c reductase core protein I
UQCRC2 ubiquinol-cytochrome c reductase core protein II
UQCRFS1 ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1
UQCRH ubiquinol-cytochrome c reductase hinge protein
UQCRHL ubiquinol-cytochrome c reductase hinge protein-like
UQCRQ ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa
VDAC1 voltage-dependent anion channel 1
VDAC2 voltage-dependent anion channel 2
VDAC3 voltage-dependent anion channel 3