Nörodejeneratif Hastalıklar Ve SUMO
Bu derlemede nörodejeneratif hastalıkların patofizyolojisinde önemi gittikçe daha da artan SUMO(Small ubiquitin-like modifiers) proteinlerinin hastalıkların mekanizmasındaki işlevleri üzerinde durulacaktır.SUMO proteinleri hücresel proseslerde önemli ölçüde rol oynamaktadır. SUMO ailesinden(memelilerde dört farklı SUMO tanımlanmıştır: SUMO1, SUMO2, SUMO3, SUMO4) herhangi birinin hedefproteinlerdeki lizin kalıntılarıyla oluşturdukları geri dönüşümlü kovalent bağlar sumolasyon işlemiolarak bilinmektedir ve sumolasyon döngüsünde meydana gelen bozulmalar birçok nörolojik bozuklukile ilişkilendirilmiştir. Huntingtin, ataksin-1, tau ve α-sinükleinin SUMO substratları olarak tanımlanmasısumolasyon ve nörodejeneratif hastalıklar arasındaki ilişkiyi destekler yöndedir. Bu derleme kapsamındailk önce SUMO proteinleri ve sumolasyon hakkında bilgi verilecek daha sonra nörodejeneratifhastalıklardan poliglutamin bozukluğuyla ilişkili hastalıkların, nöronal intranükleer inklüzyon hastalığının,Parkinson hastalığının ve Alzheimer hastalığın SUMO proteinleri ile olan ilişkisine yönelik güncelbilgiler aktarılacaktır.
Neurodegenerative Diseases andSUMO
In this review, it will be focused on the functions of SUMO (Small ubiquitin-like modifiers) proteins,which are increasingly important in the pathophysiology of neurodegenerative diseases, on the diseasemechanisms. SUMO proteins play a crucial role in cellular processes. Recyclable covalent bonds createdby one of SUMOs’ (in mammals are defined as SUMO1, SUMO2, SUMO3, SUMO4) lysin remains fromtarget proteins and impairments in the sumoylation cycle are associated with many neurological disorders.The identification of Huntingtin, ataxin-1, tau and α-synuclein as SUMO substrates leads to anassociation between sumoylation and neurodegenerative diseases. In the scope of this review, firstlyinformations about the SUMO proteins and sumoylation will be explained, thereafter informations onrelations between SUMO proteins and neurodegenerative diseases related to polyglutamine disorderrelated diseases, neuronal intranuclear inclusion disease, Parkinson’s disease and Alzheimer’s diseasewill be given.
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