Investigating satellite cell myogenic regulatory factor and Pax7 expression in children with spastic cerebral palsy

Abstract

Cerebral palsy is a group of disorders that affect movement and posture caused by disturbances to the developing brain, and is the most common motor disorder among children. 70-90% of cerebral palsy cases involve hypertonicity and shortening of the muscle and is appropriately classified as spastic cerebral palsy. Though the initial trauma that triggers cerebral palsy is usually non-progressive, the resulting disorder typically progresses and worsens over time. Because a diagnosis requires monitoring of a child’s motor abilities, cerebral palsy typically is not diagnosed until a period of time after the child begins mobilizing. Drug-based treatments and surgical approaches are employed to relieve pain and limited motion, but often have poor long-term outcomes. ☐ Studying cerebral palsy-affected muscle at the microscopic level has revealed that children with cerebral palsy have fewer skeletal muscle stem cells, known as satellite cells, in their skeletal muscle. This agrees with studies that suggest that cerebral palsy-affected muscle has a reduced regenerative capacity. It is not currently known why there are reduced numbers of satellite cells in children with cerebral palsy, or if the remaining satellite cells are functioning appropriately. The current study aimed to determine the regenerative capacity of satellite cells in cerebral palsy cases as well as their state of progression in myogenesis. This was done by employing immunohistological techniques to observe expression of transcription factors key to satellite cell function, proliferation and differentiation, including Pax7, Myf5, MyoD, and myogenin. ☐ The results of this study are currently incomplete. Unexpected expression patterns of basal lamina protein laminin make it difficult to definitively identify satellite cells. Additionally, inappropriate MyoD and myogenin signal in the sarcoplasm of myofibers makes it difficult to determine whether nuclei are expressing MyoD or myogenin. Data collection and analysis must be complete before making any solid conclusions.