Defective Ca2+ channel clustering in axon terminals

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This article discuss a defectives in calcium channels clustering in axon terminals that disturbs excitability in motoneurons resulting in spinal muscular atrophy. The article defines Proximal spinal muscular atrophy (SMA) is a motoneuron disease for which there is currently no effective treatment. SMA is essential the death of motor neurons. This disease is more common in children. This disease is process and will result in immobilization of individual who have it. The article discuss two specific genes that are associated with this disease, they are SMN1 and SMN2.

The SMN1 gene is responsible to determine the length of a protein. The SMN2 gene code for a truncated protein lacking the functionally important domains at the C terminus that are encoded by exon 7(defined by the article). SMA is caused due to lack of SMN1, causing the lack of axon elongation. In the study they noticed that mice lose most of their motoneuron abilities during the critical development period. This means that when some is born they have full motoneuron capabilities. Within the time period after birth were these motoneuron issues begin was a major focus of this experiment. They article concluded that these motoneuron problems in SMA patients are a result of differentiation of motor endplates.

This article also focus its experiment on Cyclic adenosine monophosphate (cAMP). cAMP is defined as a cyclic nucleotide, adenosine 3′,5′-cyclic monophosphate, that serves as an intracellular, and sometimes extracellular, “second messenger” mediating the action of many peptide or amine hormones. Basically means that they help to get certain hormones inside the cell; hormones like epinephrine, which that otherwise could not do the intended response or reaction. Its main purpose is of cAMP is to activate of protein kinases. Protein kinase can then modify other proteins by adding phosphate group to them commonly known as phosphorylation. The article concluded that 8-CPT cAMP enhances spontaneous Ca2+ influx into spinal motoneuronsm. Which means that this specific 8-CPT cAMP might be a possible treatment for SMA patients.

Overall, I found this article to be extremely interesting and really content rich. I think this study might give individuals suffering from SMA some hope for future research that might lead to a possible treatment. Also I was surprised that calcium plays such a huge role in disease like SMA. I definitely recommend this article 