Jason Santiago

Neuronal calcium regulation and cell stress in Malignant Hyperthermia

• Broschiertes Buch

Produktbeschreibung

Calcium ions (Ca2+) are essential signaling molecules and must be carefully regulated to preserve specificity in function. One indispensible Ca2+ signaling protein is the ryanodine receptor (RyR) calcium release channel. RyR1 is essential for muscle contraction, and RyR1mutations can lead to severe muscle disorders such as malignant hyperthermia (MH). Recently, a mouse model of human MH (Y522S-RyR1) has been developed. In skeletal muscle, Y522S-RyR1 exhibits an increased sensitivity to activation resulting in Ca2+ leak, mitochondrial disorganization, and cellular stress. Although RyR1 expression is limited in the brain, the cerebellum may be particularly vulnerable to this disease because RyR1 is highly expressed in Purkinje neurons. In this study, Y522S-RyR1 in Purkinje cells exhibits a lower threshold for activation, but does not cause severe cellular stress and damage. Future investigation of compensatory mechanisms for increased Ca2+ release in Purkinje cells could be of therapeutic value for many neurological and muscular disorders.

Produktdetails

• Verlag: LAP Lambert Academic Publishing
• Seitenzahl: 68
• Erscheinungstermin: 14. März 2016
• Englisch
• Abmessung: 220mm x 150mm x 5mm
• Gewicht: 119g
• ISBN-13: 9783659817489
• ISBN-10: 3659817481
• Artikelnr.: 44727996

Autorenporträt

Originally from the SF bay area, Jason completed undergrad at Pepperdine University. He continued his education at the University of Denver where he obtained a M.S. in Biological Sciences. He then went on to attend medical school at the University of Colorado Denver and intends to go into Child Neurology. He is happily married and has a daughter.