Is the ALS a motor neuron disease or a hematopoietic stem cell disease?

Amyotrophic lateral sclerosis (ALS) is also known as motor neuron disease (MND) or Lou Gehrig's disease. It is a fatal neurodegenerative disease the cause of which is not clear. The effective therapy is absent. ALS is diagnosed through clinical examination and neurophysiologic tests. Clinically, the symptoms manifest when about 80% of motor neurons are dead.

The hematopoietic stem cells are isolated through administration of the granulocyte colony-stimulating factor from three groups: group 1 of 62 ALS cases, group 2 of 54 ALS-free healthy donors and group 3 of 6 ALS-free ALS-family members. The expression of HLA-DR, CD38, CD117, CD13, CD33, CD56, Thy-1, CD45, СD10, CD71 was assessed in 86 samples of HSCs in ALS group, 61 samples of HSCs in healthy group and 6 samples from ALS-free ALS-family members by the multiparameter flow cytometry.

The obtained immunophenotypic profiles of HSCs membrane antigens of the ALS group significantly differ from the ALS-free group, while the immunophenotypic profiles of HSCs membrane antigens of the ALS-family members group are close to the ALS group.

We suppose that the ALS onset as the disease of HSCs and manifests in the genome and proteome of the HSCs. Such immunophenotypic profiling might permit identification of ALS-specific immune insufficiency and become a tool for early diagnostics of the ALS before clinical manifestation of the disease. New options of the updated therapy of ALS might be developed or corrected considering this new evidence.

Further research with larger samples and deeper examination is required.