Judy Benjamin

Washington, United States

Anna Serga

Russian Federation

PAPER Lalonde R, Strazielle C

PS1 knockin mice with the Japanese I213T mutation: effects on exploratory activity, motor coordination, and spatial learning.

Behav Brain Res. 2005 Jul 30;162(2):182-90. PubMed: 15908021

American Academy of Neurology 2019 Annual Meeting

CONFERENCE COVERAGE SERIES Antisense Oligonucleotides: Can They Take on ALS, SMA, Prions? Drug Reported to Help Alzheimer’s Patients Sleep Better American Academy of Neurology 2019 Annual Meeting

Antisense Oligonucleotides: Can They Take on ALS, SMA, Prions?

CONFERENCE COVERAGE 2019-05-22 Conference Coverage At the American Academy of Neurology’s 71st Annual Meeting, held May 4–10 in Philadelphia, researchers showcased their latest data on antisense oligonucleotides (ASOs). These short, synthetic, single-stranded oligodeoxynucleotides bind

Alyce Tidball

C9ORF72 Toxicity Tied to Mitochondria, Transcriptional Machinery

RESEARCH NEWS 2019-05-22 Research News Two studies offer new potential ways of quelling the neuron-killing rampage of the C9ORF72 hexanucleotide expansion, the biggest genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). In one, a mouse model fe

Laura Blair

University of South Florida
Tampa, United States

PAPER Goodman LD, Prudencio M, Kramer NJ, Martinez-Ramirez LF, Srinivasan AR, Lan M, Parisi MJ, Zhu Y, Chew J, Cook CN, Berson A, Gitler AD, Petrucelli L, Bonini NM

Toxic expanded GGGGCC repeat transcription is mediated by the PAF1 complex in C9orf72-associated FTD.

Nat Neurosci. 2019 Jun;22(6):863-874. Epub 2019 May 20 PubMed: 31110321

David Kowalski

Zheng Wang

United States

Reserve & Resilience: 1st Workshop on Research Definitions for Reserve & Resilience in Cognitive Aging and Dementia

CONFERENCE 2019-09-09 00:00:00-2019-09-10 00:00:00 Bethesda, Maryland 09 September 2019 to 10 September 2019 View Conference Website 1

Laura Ranum on C9ORF72-ALS/FTD-associated poly(GR) binds Atp5a1 and compromises mitochondrial function in vivo.

COMMENT This work provides an additional mouse model and research tool for the C9 ALS/FTD community. Because C9 ALS/FTD disease is a complex disease, with the expression of two mutant transcripts (sense and antisense) and six dipeptide repeat-containing proteins,

Robert Baloh on C9ORF72-ALS/FTD-associated poly(GR) binds Atp5a1 and compromises mitochondrial function in vivo.

COMMENT This is a very nice addition to the building literature on mechanisms of toxicity from DPR expression in animal models. It brings to light a surprisingly specific mechanism, whereby poly(GR) alters mitochondrial ATP5A1, further linking mitochondrial dynam

Adrian Isaacs on C9ORF72-ALS/FTD-associated poly(GR) binds Atp5a1 and compromises mitochondrial function in vivo.

COMMENT This paper describes an interesting new poly(GR)-expressing mouse model. We will need a range of different models to ultimately understand the role of each DPR and their contribution to C9ORF72-repeat pathology, so this is a welcome addition. The most str