Journal Article

DZNE-2026-01094

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Innate immune signaling as a potential pathomechanistic biomarker for distinct subtypes in amyotrophic lateral sclerosis.

Naumann, M. ; Kretschmer, S. ; Dorst, J.Extern* ; Lapp, H.DZNE* ; Peikert, K. ; Petri, S. ; Gess, B. ; Wolf, C. ; Rödiger, A. ; Smesny, U. ; Pan-Montojo, F. ; Kerschen, P. ; Grehl, T. ; Prudlo, J.DZNE* ; Regensburger, M. ; Ludolph, A.Extern* ; Günther, R.DZNE* ; Brenner, D.DZNE* ; Lee-Kirsch, M. A. ; Hermann, A. (Last author)DZNE*

2026
Taylor Francis Group Abingdon

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Please use a persistent id in citations: doi:10.1080/21678421.2026.2663910

Abstract: Stimulation of the innate immune system has been implicated in ALS and particularly in distinct monogenic forms of ALS. To address whether this is of diagnostic value, we performed a proof-of concept study using qPCR to assess the Interferon score in blood samples of genetic ALS. 56.5% of genetic ALS patients showed significant IFN activation, highest in C9orf72HRE patients (77.3%). About half of FUS-ALS (52.2%), but none of SOD1-ALS patients demonstrated pathological IFN scores. The IFN score significantly correlated with the ALSFRS-R slope and inversely with the time to severe event as a survival surrogate in this genetic ALS cohort. IFN + patients were more likely to be male, showed more rapid disease progression and higher neurofilament levels. The IFN score might have the potential as a stratification and readout tool for biomarker-guided individualized therapy in ALS.

Keyword(s): Humans (MeSH) ; Amyotrophic Lateral Sclerosis: immunology (MeSH) ; Amyotrophic Lateral Sclerosis: genetics (MeSH) ; Amyotrophic Lateral Sclerosis: classification (MeSH) ; Amyotrophic Lateral Sclerosis: diagnosis (MeSH) ; Amyotrophic Lateral Sclerosis: blood (MeSH) ; Male (MeSH) ; Immunity, Innate: physiology (MeSH) ; Immunity, Innate: genetics (MeSH) ; Biomarkers: blood (MeSH) ; C9orf72 Protein: genetics (MeSH) ; Female (MeSH) ; Middle Aged (MeSH) ; Signal Transduction: physiology (MeSH) ; Aged (MeSH) ; Superoxide Dismutase-1: genetics (MeSH) ; Cohort Studies (MeSH) ; Adult (MeSH) ; Disease Progression (MeSH) ; C9orf72 hexanucleotide repeat expansion ; Pathogen-associated molecular patterns (PAMPs) ; interferon score (IFN score) ; interferon type 1 ; neurofilament heavy chain ; Biomarkers ; C9orf72 Protein ; C9orf72 protein, human ; Superoxide Dismutase-1 ; SOD1 protein, human

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Contributing Institute(s):

1. Translational Neurodegeneration (AG Hermann)
2. Translational Parkinson Research (AG Falkenburger)
3. Clinical Dementia Research (Rostock /Greifswald) (AG Teipel)
4. Clinical Study Center (Ulm) (Clinical Study Center (Ulm))

Research Program(s):

1. 353 - Clinical and Health Care Research (POF4-353) (POF4-353)

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Database coverage:
; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; Ebsco Academic Search ; Essential Science Indicators ; IF < 5 ; JCR ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

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