Publications by authors named "O Ziff"
Article Synopsis
- Microglia, the brain's immune cells, are vital for neuron health but may worsen conditions like ALS and FTD, and their exact role in these diseases is still unclear.
- Researchers created specialized cultures of microglia from human stem cells with VCP mutations to study their behavior and effects on nearby nerve cells and supportive cells, using advanced techniques like RNA sequencing and proteomics.
- The studies revealed that VCP mutant microglia show immune system and lysosomal issues, react differently to inflammation compared to healthy microglia, and can influence motor neurons and astrocytes through secreted factors, even though certain genetic factors didn't fully address their dysfunction.
Article Synopsis
- ALS causes problems with a protein called TDP-43, which doesn't stay in the right part of the cell, and this affects other important materials like mRNA and proteins.
- Research using special techniques showed that motor neurons from ALS patients had major issues with misplacing mRNA and proteins, and these problems seemed connected.
- A treatment called ML240 helped fix some of these issues by helping the proteins and mRNA go back to where they should be, suggesting it could be a possible way to help treat ALS.
Amyotrophic Lateral Sclerosis (ALS) causes motor neuron degeneration, with 97% of cases exhibiting TDP-43 proteinopathy. Elucidating pathomechanisms has been hampered by disease heterogeneity and difficulties accessing motor neurons. Human induced pluripotent stem cell-derived motor neurons (iPSMNs) offer a solution; however, studies have typically been limited to underpowered cohorts.
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- SARS-CoV-2 infection can lead to various neurological problems, but the mechanisms behind this damage are not well understood.
- This study examined biomarkers in 21 patients with COVID-19 neurological syndromes compared to 23 healthy controls, focusing on signs of neuronal injury, neuroinflammation, and astrocyte activation.
- Findings indicated that patients with neurological syndromes exhibited impaired amyloid processing, increased neuronal injury markers, enhanced inflammation, and reduced astrocyte activation, suggesting a complex interplay affecting brain health in COVID-19 cases.
Amyotrophic lateral sclerosis is a rapidly progressive and fatal disease. Although astrocytes are increasingly recognized contributors to the underlying pathogenesis, the cellular autonomy and uniformity of astrocyte reactive transformation in different genetic forms of amyotrophic lateral sclerosis remain unresolved. Here we systematically examine these issues by using highly enriched and human induced pluripotent stem cell-derived astrocytes from patients with VCP and SOD1 mutations.
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