Amyotrophic lateral sclerosis (ALS) (Homo sapiens)

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MitochondriaAstrocyteEndoplasmic reticulum (ER)tBidDNA damageAggregatesElevated glutamatelevelsPPP3CCPPP3CBCYTCMKK6NEFMPPP3CANONOS1p38DAXXMAPK Signaling Pathwayp53 Signaling PathwayDNANEFHApoptosisASK1MKK3Reactive Oxygen Species (ROS)TNFalphaCa2+NONEFLPresynaptic neuronMicroglial cellMotor neuronNOL-ArginineONOO-EAAT2L-Glutamic acidL-Glutamic acidP53Neuron deathAPAF1CASP3CASP9ActivationL-Glutamic acidsynaptic transmission, glutamatergicIncrease incytosolic Ca2+Ca2+Derlin-1Accumulation ofmisfolded proteinsER stressATP depletionFall in mitochondrialmembrane potentialIncreased ROSDisturbance ofmitochondrial respirationMitochondrialdysfunctionLow Ca2+ bufferingcapacityCa2+CASP1BIDSOD1TOM40_HUMANBIM-BCL-2 complexBIM-BCL-xl complexBAXBADproteasome complexUbiquitin proteosomedysfunctionApoptosisCASP12MAP3K5TNFalphaTNFRGRIA1NONOSOD1Activation


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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
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72040view13:49, 24 October 2013SusanConnected lines
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External references


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NameTypeDatabase referenceComment
APAF1GeneProduct317 (Entrez Gene)
ASK1GeneProduct4217 (Entrez Gene)
ApoptosisPathwayWP254 (WikiPathways)
BADGeneProduct572 (Entrez Gene)
BAXGeneProduct581 (Entrez Gene)
BIDGeneProduct637 (Entrez Gene)
BIM-BCL-2 complexProteinGO:0097141 (GeneOntology)
BIM-BCL-xl complexProteinGO:0097140 (GeneOntology)
CASP12GeneProductENSG00000204403 (Ensembl)
CASP1GeneProduct834 (Entrez Gene)
CASP3GeneProduct836 (Entrez Gene)
CASP9GeneProduct842 (Entrez Gene)
CYTCProteinP01034 (Uniprot/TrEMBL)
Ca2+MetaboliteC00076 (Kegg Compound)
Ca2+MetaboliteHMDB00464 (HMDB)
DAXXGeneProduct1616 (Entrez Gene)
Derlin-1Protein79139 (Entrez Gene)
EAAT2ProteinP43004 (Uniprot/TrEMBL)
GRIA1Protein2890 (Entrez Gene)
L-ArginineMetaboliteHMDB00517 (HMDB)
L-Glutamic acidMetaboliteHMDB00148 (HMDB)
MAP3K5GeneProductENSG00000197442 (Ensembl)
MAPK Signaling PathwayPathwayWP382 (WikiPathways)
MKK3GeneProduct5605 (Entrez Gene)
MKK6GeneProduct5608 (Entrez Gene)
NEFHGeneProduct4744 (Entrez Gene)
NEFLGeneProduct4747 (Entrez Gene)
NEFMGeneProduct4741 (Entrez Gene)
NOMetaboliteHMDB03378 (HMDB)
NOS1GeneProduct4842 (Entrez Gene)
ONOO-MetaboliteHMDB02179 (HMDB)
P53ProteinP53 (Uniprot/TrEMBL)
PPP3CAGeneProduct5530 (Entrez Gene)
PPP3CBGeneProduct5532 (Entrez Gene)
PPP3CCGeneProduct5533 (Entrez Gene)
Reactive Oxygen Species (ROS)Metabolite
SOD1GeneProduct6647 (Entrez Gene)
TNFRGeneProduct7132 (Entrez Gene)
TNFalphaGeneProduct7124 (Entrez Gene)
TOM40_HUMANProteinO96008 (Uniprot/TrEMBL)
p38GeneProduct1432 (Entrez Gene)
p53 Signaling PathwayPathwayWP707 (WikiPathways)
proteasome complexProteinGO:0000502 (GeneOntology)


PathwayGO:0035249 (GeneOntology)

Annotated Interactions

No annotated interactions

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