Dataset Information

Complement profiling on sural nerves of patients with chronic inflammatory demyelinating polyneuropathy

ABSTRACT: Overall, 55 sural nerve biopsies from patients with CIDP (n=36) and CIDP-variants (n=18) were included into the study. Immunohistochemical analysis of the sural nerve specimens showed an aberrant deposition of terminal complement complex C5b-9 on endoneural capillaries in 52 (94%) of patients in addition to endoneural CD8+ T cell- and CD68+ macrophage infiltration. Gene expression studies showed an increase of factors C3 and C6 compared to NDCs with no difference in regard to clinical phenotype, whereas levels of IL6 or TNF-alpha were not significantly elevated. Our proteomic profiling approach revealed the statistically significant dysregulation of 50 proteins, which based on their respective functions suggest altered mitochondrial activity, perturbed cytoskeleton, and increased oxidative stress. The majority of patients with high to moderate complement deposition presented with a progressive disease course, however without any correlation with disease severity as measured by INCAT or MRC at baseline and follow-up. Our combined data supports the role of complement in CIDP pathogenesis. Based on these findings, we propose to further evaluate complement inhibition therapies as new targeted treatment option for patients with CIDP.

INSTRUMENT(S):

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Nerve Cord

SUBMITTER: Andreas Hentschel

LAB HEAD: Prof. Dr. Albert Sickmann

PROVIDER: PXD056286 | Pride | 2025-09-29

REPOSITORIES: Pride

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Dataset's files

Source:

			Action	DRS
	QExactiveHF02_18988_N1530.msf	Msf
	QExactiveHF02_18988_N1530.msfView	Msf
	QExactiveHF02_18988_N1530.pdResult	Other
	QExactiveHF02_18988_N1530.pdResultView	Other
	QExactiveHF02_18988_N1530.raw	Raw

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Publications

Complement profiling of sural nerves in chronic-inflammatory demyelinating polyneuropathy.

Stascheit Frauke F Roos Andreas A Schroeter Christina B CB Thomas Johanna Katrin JK Hahn Katrin K Preßler Hannah H Hentschel Andreas A Schlotter-Weigel Beate B Schoser Benedikt B Ruck Tobias T Meisel Andreas A Stenzel Werner W Preusse Corinna C

Acta neuropathologica 20250919 1

Chronic-inflammatory demyelinating polyneuropathy (CIDP) is a rare immune-mediated polyneuropathy causing substantial disability. While both cell-mediated and humoral mechanisms contribute to CIDP, the role of complement remains poorly understood. Considering the rise of complement-targeted treatment, it is crucial to examine the role of complement in CIDP. In this cross-sectional, study, sural nerve biopsies from 55 CIDP patients were analyzed using histopathology, gene- and protein-based techn ...[more]

PMID: 40971018

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