Profound downregulation of the RNA editing enzyme ADAR2 in ALS spinal motor neurons

• Published in: Neurobiology of disease Vol. 45; no. 3; pp. 1121 - 1128
• Main Authors: Hideyama, Takuto, Yamashita, Takenari, Aizawa, Hitoshi, Tsuji, Shoji, Kakita, Akiyoshi, Takahashi, Hitoshi, Kwak, Shin
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.03.2012; Elsevier
• Subjects: ADAR2 (adenosine deaminase acting on RNA 2); Adenosine Deaminase - genetics; Adenosine Deaminase - metabolism; Adult; Aged; Aged, 80 and over; ALS (amyotrophic lateral sclerosis); AMPA; Amyotrophic Lateral Sclerosis - pathology; Analysis of Variance; Animals; Bulbar Palsy, Progressive - pathology; Case-Control Studies; Down-Regulation - physiology; Female; GluA2; Humans; Male; Middle Aged; Motor Neurons - enzymology; Neurology; Neuronal death; Receptors, AMPA - genetics; Receptors, AMPA - metabolism; RNA editing; RNA-Binding Proteins - genetics; RNA-Binding Proteins - metabolism; Spinal Cord - pathology; Young Adult; CYFIP2; RNA editing; AMPA; ALS (amyotrophic lateral sclerosis); AH; ALS; ADAR2; BLCAP; Q/R; RT-PCR; PH; Neuronal death; GluA2; MSA; ADAR2 (adenosine deaminase acting on RNA 2); PBP; bladder cancer associated protein; posterior horn; glutamine/arginine; amyotrophic lateral sclerosis; AMPA receptor subunit 2; anterior horn; progressive bulbar palsy; L-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid; multiple system atrophy; reverse transcription polymerase chain reaction; cytoplasmic fragile X mental retardation protein interacting protein 2; adenosine deaminase acting on RNA 2

Amyotrophic lateral sclerosis (ALS) is the most common adult-onset fatal motor neuron disease. In spinal motor neurons of patients with sporadic ALS, normal RNA editing of GluA2, a subunit of the L-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor, is inefficient. Adenosine deamin...