A study links changes in PML nuclear bodies with TDP-43 aggregation in sporadic ALS
Original source
Relationship between promyelocytic leukemia protein nuclear bodies and TAR DNA-binding protein-43 aggregation in spinal anterior horn cells in sporadic amyotrophic lateral sclerosis. (opens in a new tab)Compass summarised this from the study's abstract.
Study details
- Studied in
- Human
Related topics
Spinal cord tissue from 12 people with sporadic amyotrophic lateral sclerosis (ALS) and five controls showed fewer promyelocytic leukemia protein nuclear bodies in motor nerve cells containing TDP-43 inclusions. The number of these nuclear bodies fell as TDP-43 inclusions became more mature. The findings also suggest an increased number of nuclear bodies in ALS cells without inclusions, which may represent an early cellular response.
Why this matters
The results add evidence that PML nuclear bodies may be involved in the cellular changes associated with sporadic ALS. This was a tissue-based study of disease mechanisms and does not yet change treatment or show that altering PML nuclear bodies would benefit people with ALS.
Limitations and context
The study examined preserved spinal cord specimens from a small group: 12 people with sporadic ALS and five controls. It used immunostaining to assess associations between PML nuclear bodies and TDP-43 inclusions, so it cannot establish cause and effect or show whether the findings apply to other forms of ALS. The work does not test a treatment or clinical outcome.