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==Overview==
==Overview==
Scientists have not found a definitive cause for ALS and the onset of the disease has been linked to several factors, including: a virus; exposure to neurotoxins or heavy metals; DNA defects; immune system abnormalities; and enzyme abnormalities. There is a known hereditary factor in familial ALS (FALS); however, there is no known hereditary component in the 90-95% cases diagnosed as sporadic ALS.
Approximately 5-10% of cases of ALS are familial ALS. More than 25 different genes could be linked to the causation of familial ALS. However, the remaining majority 90-95% of ALS cases are Sporadic ALS. There is no known cause for  Sporadic ALS.


==Cause==
==Cause==
*An inherited genetic defect linked to a defect on chromosome 21 is believed to cause approximately 40% of familial cases of ALS. This mutation is believed to be [[autosomal dominant]]
Approximately 5-10% of Familial ALS cases could be linked to different genes and their pathological pathways. More than 25 different genes mutations are attributed to Familial ALS. The most common ones include:
*The children of those diagnosed with familial ALS have a higher risk factor for developing the disease; however, those who have close family members diagnosed with sporadic ALS have no greater a risk factor than the general population [http://www.alsphiladelphia.org/pennstatehershey/newsletters/newsletter_spring04.htm]
*[[Chromosome 9 Open Reading Frame 72]](c9orf72)
*Prolonged exposure to a dietary neurotoxin is one suspected risk factor in Guam; the neurotoxin is a compound found in the seed of the [[cycad]] ''[[Cycas circinalis]]'',<ref>Bains J, et al. (2002). &quot;Isolation of various forms of sterol beta-D-glucoside from the seed of Cycas circinalis: neurotoxicity and implications for ALS-parkinsonism dementia complex&quot;.<I>J. Neurochem</I>. <B>82</B>(3):516-28. PMID: 12153476.</ref> a tropical plant found in Guam, which was used in the human food supply during the 1950s and early 1960s.
*[[TAR DNA-binding protein 43]] (TDP-43)
*[[Fused in Sarcoma]] (FUS)
*[[Superoxide Dismutase 1]] (SOD1)
*[[TANK-binding kinase 1]] (TBK1)
*[[Optineurin]] (OPTN)
 
<ref name="pmidPMID: 33918092">{{cite journal| author=Källstig E, McCabe BD, Schneider BL| title=The Links between ALS and NF-κB. | journal=Int J Mol Sci | year= 2021 | volume= 22 | issue= 8 | pages=  | pmid=PMID: 33918092 | doi=10.3390/ijms22083875 | pmc=8070122 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=33918092  }} </ref>


==References==
==References==

Latest revision as of 16:35, 21 July 2021

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

Overview

Approximately 5-10% of cases of ALS are familial ALS. More than 25 different genes could be linked to the causation of familial ALS. However, the remaining majority 90-95% of ALS cases are Sporadic ALS. There is no known cause for Sporadic ALS.

Cause

Approximately 5-10% of Familial ALS cases could be linked to different genes and their pathological pathways. More than 25 different genes mutations are attributed to Familial ALS. The most common ones include:

[1]

References

  1. Källstig E, McCabe BD, Schneider BL (2021). "The Links between ALS and NF-κB". Int J Mol Sci. 22 (8). doi:10.3390/ijms22083875. PMC 8070122 Check |pmc= value (help). PMID 33918092 PMID: 33918092 Check |pmid= value (help).

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