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Amyotrophic lateral sclerosis (ALS) - Ailuropoda melanoleuca

 

Model information
Identifier: BMID000000004617
Format: SBML L3 V1 (Layout, Qualitative Models)
Project: path2models
Categories: non-metabolic
Submission: 17 May 2012 14:41:02 UTC
Last modified: 07 Dec 2012 20:46:19 UTC
Published: 19 May 2012 23:49:21 UTC
Annotations
occursIn Ailuropoda melanoleuca Taxonomy
isDerivedFrom Amyotrophic lateral sclerosis (ALS) KEGG Pathway
Notes
Model of “Amyotrophic lateral sclerosis (ALS)” in “Ailuropoda melanoleuca (giant panda)”
Amyotrophic lateral sclerosis (ALS) is a progressive, lethal, degenerative disorder of motor neurons. The hallmark of this disease is the selective death of motor neurons in the brain and spinal cord, leading to paralysis of voluntary muscles. Mutant superoxide dismutase 1 (SOD1), as seen in some familial ALS (FALS) cases, is unstable, forming aggregates in the motor neuron cytoplasm, axoplasm and mitochondria. Within mitochondria, mutant SOD1 may interfere with the anti-apoptotic function of Bcl-2, affect mitochondrial import by interfering with the translocation machinery (TOM/TIM), and generate toxic free radicals (ROS). Reactive oxygen species (ROS), produced within mitochondria, inhibit the function of EAAT2, the main glial glutamate transporter protein, responsible for most of the reuptake of synaptically released glutamate. Glutamate excess increases intracellular calcium, which enhances oxidative stress and mitochondrial damage. Mutant SOD1 can also trigger oxidative reactions , which can then cause damage through the formation of hydroxyl radicals or via nitration of tyrosine residues on proteins. Nitration may target neurofilament proteins, affecting axonal transport. Collectively, these mechanisms are predicted to disturb cellular homeostasis, ultimately triggering motor neuron death.

Graphical representation of 'Amyotrophic lateral sclerosis (ALS) (Ailuropoda melanoleuca (giant panda))'
(PNG image hosted by the Kyoto Encyclopedia of Genes and Genomes, KEGG).
This model has been automatically generated by KEGGtranslator V2.3.0 (KEGGtranslator: visualizing and converting the KEGG PATHWAY database to various formats. Wrzodek C, Dräger A, Zell A. Bioinformatics . 2011, 27 :2314-2315) using information coming from the KEGG PATHWAY Database ( original pathway ).
This model has been produced by the path2models project, it is currently hosted on BioModels Database and identified by: BMID000000004617 .
To the extent possible under law, all copyright and related or neighbouring rights to this encoded model have been dedicated to the public domain worldwide. Please refer to CC0 Public Domain Dedication for more information.
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