التفاصيل البيبلوغرافية
| العنوان: |
Video_2_Axon-Specific Mitochondrial Pathology in SPG11 Alpha Motor Neurons.MP4 |
| المؤلفون: |
Fabian Güner (11086335), Tatyana Pozner (6055349), Florian Krach (11086338), Iryna Prots (230682), Sandra Loskarn (11086341), Ursula Schlötzer-Schrehardt (283317), Jürgen Winkler (208179), Beate Winner (602511), Martin Regensburger (617357) |
| سنة النشر: |
2021 |
| المجموعة: |
Smithsonian Institution: Digital Repository |
| مصطلحات موضوعية: |
Neuroscience, Biological Engineering, Developmental Biology, Stem Cells, Artificial Intelligence and Image Processing, Endocrinology, Radiology and Organ Imaging, Autonomic Nervous System, Cellular Nervous System, Central Nervous System, Sensory Systems, Clinical Nursing: Tertiary (Rehabilitative), Decision Making, Rehabilitation Engineering, Biomedical Engineering not elsewhere classified, Signal Processing, Neurogenetics, Image Processing, SPG11, hereditary spastic paraplegia, alpha motor neuron, induced pluripotent stem cells, mitochondria, axonal transport |
| الوصف: |
Pathogenic variants in SPG11 are the most frequent cause of autosomal recessive complicated hereditary spastic paraplegia (HSP). In addition to spastic paraplegia caused by corticospinal degeneration, most patients are significantly affected by progressive weakness and muscle wasting due to alpha motor neuron (MN) degeneration. Mitochondria play a crucial role in neuronal health, and mitochondrial deficits were reported in other types of HSPs. To investigate whether mitochondrial pathology is present in SPG11, we differentiated MNs from induced pluripotent stem cells derived from SPG11 patients and controls. MN derived from human embryonic stem cells and an isogenic SPG11 knockout line were also included in the study. Morphological analysis of mitochondria in the MN soma versus neurites revealed specific alterations of mitochondrial morphology within SPG11 neurites, but not within the soma. In addition, impaired mitochondrial membrane potential was indicative of mitochondrial dysfunction. Moreover, we reveal neuritic aggregates further supporting neurite pathology in SPG11. Correspondingly, using a microfluidic-based MN culture system, we demonstrate that axonal mitochondrial transport was significantly impaired in SPG11. Overall, our data demonstrate that alterations in morphology, function, and transport of mitochondria are an important feature of axonal dysfunction in SPG11 MNs. |
| نوع الوثيقة: |
dataset |
| اللغة: |
unknown |
| العلاقة: |
https://figshare.com/articles/media/Video_2_Axon-Specific_Mitochondrial_Pathology_in_SPG11_Alpha_Motor_Neurons_MP4/14921460Test |
| DOI: |
10.3389/fnins.2021.680572.s005 |
| الإتاحة: |
https://doi.org/10.3389/fnins.2021.680572.s005Test |
| حقوق: |
CC BY 4.0 |
| رقم الانضمام: |
edsbas.EC7FCCC2 |
| قاعدة البيانات: |
BASE |
