is our passion
Home / Projects / Frontotemporal Lobar Degeneration

Frontotemporal Lobar Degeneration

NOMIS Project 2016

— 2022

The German Center for Neurodegenerative Diseases (Deutsches Zentrum für Neurodegenerative Erkrankungen, DZNE) is committed to understanding commonalities and differences between various brain diseases in order to develop new preventive and therapeutic approaches. The Frontotemporal Lobar Degeneration – from the Basic Mechanism and Target Identification to Translational and Clinical Approaches project is based on a new, cross-cutting approach: clinical and basic research are done at the same time, which allows their results to be directly fed into each other’s processes. NOMIS is supporting this innovative framework by matching DZNE funding for the clinical research with funding for the basic research component; this will focus on identifying therapeutic targets and understanding their physiological function, as well as finding the mechanism through which mutant genes cause the disease. The Frontotemporal Lobar Degeneration project comprises the search for biomarkers as well as screening for disease-modulating compounds.

The project is being led by Christian Haass and Dieter Edbauer at the DZNE.


NOMIS Researcher(s)

Member of the NOMIS Foundation Board of Directors
German Center for Neurodegenerative Diseases (DZNE), NOMIS Foundation

Project News

NOMIS Board Member and researcher Christian Haass has been awarded the 2022 Hector Wissenschaftspreis (science award) for his groundbreaking research on Alzheimer’s disease, his interdisciplinary approach as a university professor, […]


Project Insights

Abstract: The expanded hexanucleotide GGGGCC repeat mutation in the C9orf72 gene is the main genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. Under one disease mechanism, sense and antisense transcripts of the repeat are predicted to bind various RNA-binding proteins, compromise their function and cause cytotoxicity. Here we identify phenylalanine-tRNA
Abstract: Purpose: Early after [18F]PI-2620 PET tracer administration, perfusion imaging has potential for regional assessment of neuronal injury in neurodegenerative diseases. This is while standard late-phase [18F]PI-2620 tau-PET is able to discriminate the 4-repeat tauopathies progressive supranuclear palsy and corticobasal syndrome (4RTs) from disease controls and healthy controls. Here, we investigated
Abstract: Aims: Psychotic symptoms are increasingly recognized as a distinguishing clinical feature in patients with dementia due to frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP). Within this group, carriers of the C9orf72 repeat expansion are particularly prone to develop delusions and hallucinations. Methods: The present retrospective study sought to provide novel
Abstract: Motoneurons are one of the most energy-demanding cell types and a primary target in Amyotrophic lateral sclerosis (ALS), a debilitating and lethal neurodegenerative disorder without currently available effective treatments. Disruption of mitochondrial ultrastructure, transport, and metabolism is a commonly reported phenotype in ALS models and can critically affect survival and
Abstract: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder causing progressive loss of motor neurons. Mutations in Fused in sarcoma (FUS) leading to its cytoplasmic mislocalization cause a subset of ALS. Under stress, mutant FUS localizes to stress granules (SGs)—cytoplasmic condensates composed of RNA and various proteins. Aberrant dynamics of SGs
Abstract: An international consensus report in 2019 recommended a classification system for limbic-predominant age-related TDP-43 encephalopathy neuropathologic changes (LATE-NC). The suggested neuropathologic staging system and nomenclature have proven useful for autopsy practice and dementia research. However, some issues remain unresolved, such as cases with unusual features that do not fit with