Neurodegenerative Diseases andSUMO

Yıl 2017, Cilt: 7 Sayı: 13, 59 - 70, 01.06.2017

Ulaş Ay Melis Şen Ece Akbayır Erdem Tüzün , Cem İsmail Küçükali

Öz

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 disease

mechanisms. SUMO proteins play a crucial role in cellular processes. Recyclable covalent bonds created

by one of SUMOs’ (in mammals are defined as SUMO1, SUMO2, SUMO3, SUMO4) lysin remains from

target 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 an

association between sumoylation and neurodegenerative diseases. In the scope of this review, firstly

informations about the SUMO proteins and sumoylation will be explained, thereafter informations on

relations between SUMO proteins and neurodegenerative diseases related to polyglutamine disorder

related diseases, neuronal intranuclear inclusion disease, Parkinson’s disease and Alzheimer’s disease

will be given.

Anahtar Kelimeler

SUMO and sumoylation, Polyglutamine diseases, Alzheimer diseases, Parkinson’s disease

Nörodejeneratif Hastalıklar Ve SUMO

Öz

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 hedef

proteinlerdeki lizin kalıntılarıyla oluşturdukları geri dönüşümlü kovalent bağlar sumolasyon işlemi

olarak bilinmektedir ve sumolasyon döngüsünde meydana gelen bozulmalar birçok nörolojik bozukluk

ile ilişkilendirilmiştir. Huntingtin, ataksin-1, tau ve α-sinükleinin SUMO substratları olarak tanımlanması

sumolasyon ve nörodejeneratif hastalıklar arasındaki ilişkiyi destekler yöndedir. Bu derleme kapsamında

ilk önce SUMO proteinleri ve sumolasyon hakkında bilgi verilecek daha sonra nörodejeneratif

hastalıklardan poliglutamin bozukluğuyla ilişkili hastalıkların, nöronal intranükleer inklüzyon hastalığının,

Parkinson hastalığının ve Alzheimer hastalığın SUMO proteinleri ile olan ilişkisine yönelik güncel

bilgiler aktarılacaktır.

Anahtar Kelimeler

SUMO ve sumolasyon, Poliglutamin hastalıklar, Alzheimer hastalığı, Parkinson hastalığı

Kaynakça

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