Dataset Information

Proteomics of Duchenne Muscular Dystrophy Patient iPSC - Derived Skeletal Muscle Cells Reveal Differential Expression of Myosin 9, Collagen 18A, Tropomyosin 1, NCAM1, BASP1 and RUVBL1

ABSTRACT: Proteomics of skeletal muscular dystrophies is limited by the amount of protein which can be provided from patient biopsies. Cells and tissues derived from patient induced pluripotent stem cells (iPSCs) can be an expandable alternative source. We have patterned iPSCs from three Du-chenne Muscular Dystrophy (DMD) patient lines into skeletal muscle cells using a 2-dimensional as well as our 3-dimensional organoid differentiation system. Probes with sufficient protein amounts could be extracted and prepared for mass spectrometry. In total, 3007 proteins in 2D and 2709 proteins in 3D were detected in DMD patient probes. 83 proteins in 2D and 338 proteins in 3D can be described as differentially expressed between DMD and control patient probes in a post-hoc-Test. We have identified and propose Myosin-9, Collagen 18A, Tropomyosin 1, BASP1, RUVBL1 and NCAM1 as proteins specifically altered in their expression in DMD for further in-vestigations. Proteomics of skeletal muscle organoids resulted in greater consistency of results between cell lines in comparison to the 2-dimensional myogenic differentiation protocol.

INSTRUMENT(S):

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Pluripotent Stem Cell, Skeletal Muscle Myoblast In Skeletal Muscle

DISEASE(S): Duchenne Muscular Dystrophy

SUBMITTER: Britta Eggers

LAB HEAD: Katrin Marcus

PROVIDER: PXD068000 | Pride | 2025-11-17

REPOSITORIES: Pride

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Publications

Proteomics of Duchenne Muscular Dystrophy Patient iPSC-Derived Skeletal Muscle Cells Reveal Differential Expression of Cytoskeletal and Extracellular Matrix Proteins.

Gallert Sarah-Marie SM Fölsch Mitja M Mavrommatis Lampros L Kindler Urs U Schork Karin K Eisenacher Martin M Vorgerd Matthias M Brand-Saberi Beate B Eggers Britta B Marcus Katrin K Zaehres Holm H

Cells 20251028 21

Proteomics of dystrophic muscle samples is limited by the amount of protein that can be extracted from patient biopsies. Cells and tissues derived from patient-derived induced pluripotent stem cells (iPSCs) can be an expandable alternative source. We have patterned iPSCs from three Duchenne muscular dystrophy (DMD) patient lines into skeletal muscle cells using a two-dimensional as well as our three-dimensional organoid differentiation system. Probes with sufficient protein amounts could be extr ...[more]

PMID: 41227334

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