Amyotrophic lateral sclerosis (ALS) (Homo sapiens)

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MitochondriaEndoplasmic reticulum (ER)AstrocyteATP depletionFall in mitochondrial membrane potentialMitochondriaReduced expressionDNA damageIncreased ROSElevated glutamatelevelsAggregatesAggregatesMitochondrial dysfunctionNeurofilament damagetBidLow Ca2+ buffering capacityIncreased expressionImpairment of axonal transportAbnormal Rab5 dependent endycytosis?MKK6MAPK Signaling PathwayApoptosisTNFalphaNOS1RAB5ANEFMProteasomePPP3CCCYTCReactive Oxygen Species (ROS)MKK3ASK1PRPHDNACATOHO2-PPP3CAH2Op53 Signaling PathwayPPP3CBNEFHH2O2NOCCSNEFLNOALS2GPX1Ca2+p38RAC1CuDAXXPresynaptic neuronMicroglial cellMotor neuronNOL-ArginineONOO-EAAT2L-Glutamic acidL-Glutamic acidP53Neuron deathAPAF1CASP3CASP9ActivationL-Glutamic acidsynaptic transmission, glutamatergicIncrease incytosolic Ca2+Ca2+Derlin-1Accumulation ofmisfolded proteinsER stressDisturbance ofmitochondrial respirationCASP1BIDSOD1TOM40BCL2BCL2L1BAXBADUbiquitin proteosomedysfunctionCASP12TNFalphaTNFRGRIA1NONOSOD1ActivationCa2+ASK1ApoptosisReactive Oxygen Species (ROS)SOD1SOD1


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Ontology Terms



  1. Flomen R, Makoff A; ''Increased RNA editing in EAAT2 pre-mRNA from amyotrophic lateral sclerosis patients: involvement of a cryptic polyadenylation site.''; Neurosci Lett, 2011 PubMed Europe PMC Scholia


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113794view09:07, 24 November 2020Finterlysmall graphical update
113792view08:56, 24 November 2020Finterlymake pathways clickable
113789view17:09, 23 November 2020FinterlySmall graphical update and fixed links
109207view21:31, 26 February 2020KhanspersAdded mutation states
108062view13:19, 27 November 2019FehrhartOntology Term : 'amyotrophic lateral sclerosis' added !
106714view13:15, 17 September 2019MaintBotHMDB identifier normalization
105880view04:52, 16 August 2019KhanspersModified description
105527view06:00, 9 August 2019KhanspersModified description
96316view09:07, 8 March 2018Mkutmonremove old WP category
96258view16:25, 2 March 2018EgonwOntology Term : 'disease pathway' added !
96111view11:20, 18 February 2018EgonwReplaced mim-conversion with arrow for things that are not conversions.
96110view11:13, 18 February 2018EgonwMade a number of labels readable (again)
96105view14:38, 16 February 2018DeSlChanged MIMconversion to arrow between gene and metabolite
85186view14:39, 23 April 2016EgonwReplaced a mim-conversion with Arrow.
85057view10:52, 10 April 2016EgonwReplaced a Wikipedia ID with a ChEBI ID.
85049view10:22, 10 April 2016EgonwReplaced a Wikipedia ID with a ChEBI ID.
84415view21:47, 23 February 2016AlexanderPicoQuick edit to datanode annotation or property
79862view12:17, 21 April 2015Mkutmonfixed linkout to wikipedia for ROS
75221view21:48, 9 May 2014Khansperschnaged DNA from gene product to label
72245view20:53, 29 October 2013Khansperschanged pathway node xrefs from GO to WP
72040view13:49, 24 October 2013SusanConnected lines
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External references


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NameTypeDatabase referenceComment
ALS2GeneProduct57679 (Entrez Gene)
APAF1GeneProduct317 (Entrez Gene)
ASK1GeneProduct4217 (Entrez Gene)
ApoptosisPathwayWP254 (WikiPathways)
BADGeneProduct572 (Entrez Gene)
BAXGeneProduct581 (Entrez Gene)
BCL2GeneProduct596 (Entrez Gene)
BCL2L1GeneProduct598 (Entrez Gene)
BIDGeneProduct637 (Entrez Gene)
CASP12GeneProductENSG00000204403 (Ensembl)
CASP1GeneProduct834 (Entrez Gene)
CASP3GeneProduct836 (Entrez Gene)
CASP9GeneProduct842 (Entrez Gene)
CATGeneProduct847 (Entrez Gene)
CCSGeneProduct9973 (Entrez Gene)
CYTCProteinP01034 (Uniprot/TrEMBL)
Ca2+MetaboliteC00076 (Kegg Compound)
Ca2+MetaboliteHMDB00464 (HMDB)
CuMetaboliteHMDB00657 (HMDB)
DAXXGeneProduct1616 (Entrez Gene)
Derlin-1Protein79139 (Entrez Gene)
EAAT2ProteinP43004 (Uniprot/TrEMBL)
GPX1GeneProduct2876 (Entrez Gene)
GRIA1Protein2890 (Entrez Gene)
H2O2MetaboliteHMDB03125 (HMDB)
H2OMetaboliteHMDB02111 (HMDB)
L-ArginineMetaboliteHMDB00517 (HMDB)
L-Glutamic acidMetaboliteHMDB00148 (HMDB)
MAPK Signaling PathwayPathwayWP382 (WikiPathways)
MKK3GeneProduct5605 (Entrez Gene)
MKK6GeneProduct5608 (Entrez Gene)
NEFHGeneProduct4744 (Entrez Gene)
NEFLGeneProduct4747 (Entrez Gene)
NEFMGeneProduct4741 (Entrez Gene)
NOMetaboliteHMDB03378 (HMDB)
NOS1GeneProduct4842 (Entrez Gene)
O2-MetaboliteHMDB02168 (HMDB)
OHMetabolite96023368 (PubChem-substance)
ONOO-MetaboliteHMDB02179 (HMDB)
P53ProteinP53 (Uniprot/TrEMBL)
PPP3CAGeneProduct5530 (Entrez Gene)
PPP3CBGeneProduct5532 (Entrez Gene)
PPP3CCGeneProduct5533 (Entrez Gene)
PRPHGeneProduct5630 (Entrez Gene)
ProteasomePathwayWP183 (WikiPathways)
RAB5AGeneProduct5868 (Entrez Gene)
RAC1GeneProduct5879 (Entrez Gene)
Reactive Oxygen Species (ROS)Metabolite
SOD1GeneProduct6647 (Entrez Gene)
TNFRGeneProduct7132 (Entrez Gene)
TNFalphaGeneProduct7124 (Entrez Gene)
TOM40ProteinO96008 (Uniprot/TrEMBL)
p38GeneProduct1432 (Entrez Gene)
p53 Signaling PathwayPathwayWP707 (WikiPathways)


PathwayGO:0035249 (GeneOntology)

Annotated Interactions

No annotated interactions

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