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| 037 | _ | _ | |a DZNE-2025-01377 |
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| 100 | 1 | _ | |a Heger, Leonie |0 P:(DE-2719)9001931 |b 0 |u dzne |
| 245 | _ | _ | |a Patient-Derived Neurons Exhibit α-Synuclein Pathology and Previously Unrecognized Major Histocompatibility Complex Class I Elevation in Mitochondrial Membrane Protein-Associated Neurodegeneration. |
| 260 | _ | _ | |a New York, NY |c 2025 |b Wiley |
| 336 | 7 | _ | |a article |2 DRIVER |
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| 520 | _ | _ | |a Mitochondrial membrane protein-associated neurodegeneration (MPAN) from the neurodegeneration with brain iron accumulation (NBIA) family is a rare neurodegenerative disease marked by α-synuclein aggregation, brain iron accumulation, and midbrain dopaminergic neuron degeneration.The mechanisms driving neuron vulnerability remain unclear. Our study aimed to develop a patient-derived disease model replicating key pathologies of patient brains.We generated induced pluripotent stem cell-derived midbrain dopaminergic neurons from MPAN patients and examined ultrastructural and biochemical markers of pathology.MPAN patient neurons displayed α-synuclein aggregation, axonal swellings, iron accumulation, and severe membrane destruction. In addition, levels of the major histocompatibility complex class I (MHC-I), linked to cellular stress and neurodegenerative processes, were elevated in patient neurons. Treatment with acetyl-leucine, a potentially neuroprotective compound, decreased MHC-I.This first patient-derived neuronal model of MPAN provides a useful tool for further research aimed at unraveling the complexities of this disease and developing potential therapeutic interventions. © 2025 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society. |
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| 650 | _ | 7 | |a MPAN |2 Other |
| 650 | _ | 7 | |a NBIA |2 Other |
| 650 | _ | 7 | |a dopaminergic neurons |2 Other |
| 650 | _ | 7 | |a iPSC disease modeling |2 Other |
| 650 | _ | 7 | |a α‐synuclein |2 Other |
| 700 | 1 | _ | |a Kertess, Leonie |b 1 |
| 700 | 1 | _ | |a Kaufhold, Clara Margarete |0 P:(DE-2719)9002163 |b 2 |u dzne |
| 700 | 1 | _ | |a Gubinelli, Francesco |0 P:(DE-2719)9002293 |b 3 |u dzne |
| 700 | 1 | _ | |a Cardona-Alberich, Aida |b 4 |
| 700 | 1 | _ | |a Özata, Gamze |b 5 |
| 700 | 1 | _ | |a Müller, Stephan A |0 P:(DE-2719)2810938 |b 6 |
| 700 | 1 | _ | |a Tschirner, Sarah K |0 P:(DE-2719)9000794 |b 7 |u dzne |
| 700 | 1 | _ | |a Stehling, Oliver |b 8 |
| 700 | 1 | _ | |a Schifferer, Martina |0 P:(DE-2719)2812260 |b 9 |u dzne |
| 700 | 1 | _ | |a Peron, Camille |b 10 |
| 700 | 1 | _ | |a Tiranti, Valeria |b 11 |
| 700 | 1 | _ | |a Lill, Roland |b 12 |
| 700 | 1 | _ | |a Iuso, Arcangela |b 13 |
| 700 | 1 | _ | |a Zecca, Luigi |b 14 |
| 700 | 1 | _ | |a Strupp, Michael |b 15 |
| 700 | 1 | _ | |a Oertel, Wolfgang |0 P:(DE-2719)9000908 |b 16 |
| 700 | 1 | _ | |a Lichtenthaler, Stefan F |0 P:(DE-2719)2181459 |b 17 |u dzne |
| 700 | 1 | _ | |a Burbulla, Lena F. |0 P:(DE-2719)9000040 |b 18 |e Last author |u dzne |
| 773 | _ | _ | |a 10.1002/mds.70029 |g Vol. 40, no. 12, p. 2811 - 2818 |0 PERI:(DE-600)2041249-6 |n 12 |p 2811 - 2818 |t Movement disorders |v 40 |y 2025 |x 0885-3185 |
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