A cognizance analysis on amyotrophic lateral sclerosis in Middle East countries

Abstract

ALS, or amyotrophic lateral sclerosis, is a progressive neurodegenerative disease, affects nerve cells in the brain and spinal cord [1]. Ever since, the repeat expansions in the C9ORF72 gene were identified as a major cause of ALS and frontotemporal dementia, researchers have been debating whether the disease is caused by loss of normal protein(s) or accumulation of toxic aggregates [2]. Amyotrophic lateral sclerosis (ALS) epidemiology has rapidly developed in the Middle East, was spread after the gulf war [3]. In 2015, there were 45,810 men and 34,352 women diagnosed with ALS in the developed countries. The projected number ALS is expected to grow from 80,162 in 2015 to 105,693 in 2040, represents an increase of >31%. In Middle East (like, Iran) there is high prevalence in ALS, 112% (Fig-1), due to insignificant awareness on ALS [4]. The recent discovery of pathological TDP-43 in both ALS and FTLD-U confirms a new biochemical class neurodegenerative disease, called TDP-43 proteinopathies [5]. Recently, researchers developed an antibody that reduces the number of TDP-43 protein aggregates in the mice brains with ALS, resulting in significant improvements in their cognitive and motor performance (Fig-2). Preliminary results suggest that injecting TDP-43 antibodies directly into the cerebrospinal fluid could effectively reduce protein aggregates in nerve cells [6]. The current cognizance analysis focus on the mechanism of single-chain (scFv) antibodies against the RNA recognition motif 1 (RRM1) of TDP-43 protein aggregates in the ALS pathology.