Reactive Neuroblastosis in Huntington’s Disease: A Putative Therapeutic Target for Striatal Regeneration in the Adult Brain

Front. Cell. Neurosci., 2018 · DOI: 10.3389/fncel.2018.00037 · Published: March 9, 2018

Simple Explanation

The review proposes that the abnormal production of neuroblasts in the striatum of Huntington's disease (HD) brains, termed "reactive neuroblastosis," could be a self-repair mechanism. This process might support tissue regeneration to compensate for structural and physiological deficits caused by aging or the neurodegenerative process in the striatum. The authors discuss the regulation of striatal neurogenesis and neuroblastosis, considering their functional relevance in the context of HD.

Study Duration

Not specified

Participants

Animal models of Huntington’s disease (HD) and in post-mortem brain of HD patients

Evidence Level

Level 5, Hypothesis and Theory

Key Findings

1
Reactive neuroblastosis, characterized by the abnormal proliferation and migration of neuroblasts towards the degenerating striatum, is observed in HD brains.
2
The process of neurogenesis seems to be prematurely terminated in reactive neuroblastosis, with cells dying before maturation and integration into the striatal circuitry.
3
Elevated levels of TGF-beta in the HD brain may contribute to reduced NSC activity and premature death of differentiating neuroblasts.

Research Summary

The review focuses on the reciprocal relationship between adult neurogenesis, cognitive, and motor functions in the context of neurodegenerative disorders like Huntington's disease (HD). It introduces the concept of "reactive neuroblastosis" in HD, characterized by increased neuroblast production and migration towards the striatum, potentially representing a self-repair mechanism. The authors discuss the potential role of TGF-beta signaling and mutant huntingtin protein in regulating neurogenesis and neuroblast survival in the HD striatum.

Practical Implications

Therapeutic Target Identification

Reactive neuroblastosis could be a potential therapeutic target for striatal regeneration in HD.

Understanding Disease Mechanisms

Further investigation into the molecular mechanisms of reactive neuroblastosis may reveal insights into HD pathogenesis.

Novel Therapeutic Strategies

Modulating TGF-beta signaling or promoting neuroblast survival could offer new therapeutic avenues for HD and other neurological disorders.

Study Limitations

1
The precise functional relevance of reactive neuroblastosis remains unknown.
2
Current techniques for detecting and analyzing neurogenesis have limitations.
3
The role of striatal neuroblasts in normal human brains requires further elucidation.

Your Feedback

Was this summary helpful?

Back to Regenerative Medicine