![]() 3,9 In FFI, these prions build up mostly within the thalamus of the brain. 7Īs mentioned previously, the exact function of misfolded prion proteins remains unclear, but its impact is knowingly toxic to the body. 7 This mutation occurs on codon 178 at the prion-related protein (PRPN) gene 7 Here, normal aspartic acid (Asp) is substituted for asparagine (Asn), causing abnormalities and the creation of a misfolded prion protein. 1įFI is often inherited through an autosomal dominant pattern, which means that one copy of the mutation passed on from parent to offspring is enough to cause the disease. Some more commonly heard examples of prion disease include Creutzfeldt-Jakob disease and bovine spongiform encephalopathy (also known as “mad cow disease”). What makes the prion protein particularly perilous is its unfortunate property of often being misfolded into abnormal conformations and accumulating in the brain. However, the misfolding of proteins typically denotes the creation of an unwanted function and/or the loss of an important function. 3,9 The function of the prion protein, normal or misfolded, is currently unclear. In addition to its rarity, FFI is noteworthy as it is a disease that lands in the dangerous half of the insomnia spectrum, with most of its patients dying of the disease only a few years following initial diagnosis.įFI is a prion disease, which is a branch of neurodegenerative disorders caused by misfolded prion proteins.ģ More specifically, FFI belongs within the transmissible spongiform encephalopathies category. There are only about 57 reported cases of FFI that exist in 27 familial lines. While insomnia is a widely known disorder, there is an existing rare genetic variant of insomnia, known as Fatal Familial Insomnia (FFI). With the loss or absence of sleep, insomniacs find their own livelihood slowly wither away with the worsening of proper physical, social and mental functioning.Īs such, insomnia is an incredibly dangerous disorder. In the case of insomnia, there are those who simply struggle to sleep, and others that may stay wide awake for days on end, falling into a descent of hallucinations rather than falling asleep. One such instance is insomnia, a sleep disorder that affects the lives of approximately 30% of the adult population. However, there are instances where the choice between sleep and no sleep is not a conscious decision. Fortunately, for most, a few days of improper sleep is likely impermanent and only a result of temporary circumstances. ![]() Regardless, performing day-to-day tasks after consecutive days without proper sleep is often debilitating. With humans, for instance, the inability to attain a good night's rest might stem from various life stressors, health conditions or even too much caffeine. Unfortunately, sleep doesn't always come easily. To virtually all species, sleep is integral for survival and good health.
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |