Yıl 2020, Cilt 13 , Sayı 2, Sayfalar 477 - 484 2020-05-14

Pathogenesis of Amyotrophic Lateral Sclerosis (ALS)
Amyotrofik Lateral Skleroz (ALS) Hastalığının Patogenezi

Ahmet Doğucem MARANGOZ [1] , Çağdaş ERDOĞAN [2]


Amyotrophic Lateral Sclerosis (ALS), first described by Charcot in the nineteenth century, is a progressive neurodegenerative disease, usually with a three to five-year survival. ALS is characterized by degeneration and death in motor neurons. Cortical motor cells (pyramidal and Betz cells), corticospinal tract and anterior horn cells show prominent axonal loss. Although the etiology of ALS is not known exactly, it is suggested that many different factors play a role in its pathogenesis. Genetics, oxidative stress, glutamate excitotoxicity, mitochondrial dysfunction, axonal transport disorder, neuroinflammation, RNA disorders are the main ones. Factors such as neurotrophic factors, impaired intercellular traffic, disturbance of signaling pathways and metabolic changes are thought to play a role in the process. In this article, pathogenesis of ALS disease is reviewed.

İlk olarak on dokuzuncu yüzyılda Charcot tarafından tanımlanan  Amyotrofik Lateral Skleroz (ALS), genellikle üç ile beş yıllık bir sağkalım ile seyreden, sürekli ilerleyici bir nörodejeneratif hastalıktır. Motor nöronlarda dejenerasyon ve ölüm ile karakterize olup,  kortikal motor hücreler(piramidal ve Betz hücreleri), kortikospinal trakt, ve ön boynuz hücrelerinde belirgin aksonal kayıp görülür. ALS’nin etiyolojisi tam olarak bilinmemekle birlikte, patogenezinde pek çok farklı etmenin rol oynadığı ileri sürülmektedir. Genetik, oksidatif stres, glutamat eksitoksisitesi, mitokondrial disfonksiyon, aksonal transport bozukluğu, nöroinflamasyon, RNA bozuklukları bunlardan başlıcalarıdır. Yine nörotrofik faktörler, organeller arası trafiğin bozulması, sinyal yolaklarında bozukluk, metabolik değişiklikler gibi faktörlerin de süreçte rol oynadığı düşünülmektedir. Bu derlemede ALS hastalığının patogenezi gözden geçirilmiştir.

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Birincil Dil tr
Konular Klinik Nöroloji
Yayımlanma Tarihi Mayıs 2020
Bölüm Derleme
Yazarlar

Orcid: 0000-0001-7647-3435
Yazar: Ahmet Doğucem MARANGOZ (Sorumlu Yazar)
Kurum: PAMUKKALE ÜNİVERSİTESİ, TIP FAKÜLTESİ
Ülke: Turkey


Orcid: 0000-0002-6231-7834
Yazar: Çağdaş ERDOĞAN
Kurum: PAMUKKALE ÜNİVERSİTESİ, TIP FAKÜLTESİ
Ülke: Turkey


Tarihler

Yayımlanma Tarihi : 14 Mayıs 2020

Bibtex @derleme { patd643460, journal = {Pamukkale Tıp Dergisi}, issn = {}, eissn = {1308-0865}, address = {Pamukkale Üniversitesi Tıp Fakültesi Eğitim Blokları Kınıklı kampüsü 20070 Kınıklı, Denizli}, publisher = {Pamukkale Üniversitesi}, year = {2020}, volume = {13}, pages = {477 - 484}, doi = {10.31362/patd.643460}, title = {Amyotrofik Lateral Skleroz (ALS) Hastalığının Patogenezi}, key = {cite}, author = {MARANGOZ, Ahmet Doğucem and ERDOĞAN, Çağdaş} }
APA MARANGOZ, A , ERDOĞAN, Ç . (2020). Amyotrofik Lateral Skleroz (ALS) Hastalığının Patogenezi. Pamukkale Tıp Dergisi , 13 (2) , 477-484 . DOI: 10.31362/patd.643460
MLA MARANGOZ, A , ERDOĞAN, Ç . "Amyotrofik Lateral Skleroz (ALS) Hastalığının Patogenezi". Pamukkale Tıp Dergisi 13 (2020 ): 477-484 <https://dergipark.org.tr/tr/pub/patd/issue/54327/643460>
Chicago MARANGOZ, A , ERDOĞAN, Ç . "Amyotrofik Lateral Skleroz (ALS) Hastalığının Patogenezi". Pamukkale Tıp Dergisi 13 (2020 ): 477-484
RIS TY - JOUR T1 - Amyotrofik Lateral Skleroz (ALS) Hastalığının Patogenezi AU - Ahmet Doğucem MARANGOZ , Çağdaş ERDOĞAN Y1 - 2020 PY - 2020 N1 - doi: 10.31362/patd.643460 DO - 10.31362/patd.643460 T2 - Pamukkale Tıp Dergisi JF - Journal JO - JOR SP - 477 EP - 484 VL - 13 IS - 2 SN - -1308-0865 M3 - doi: 10.31362/patd.643460 UR - https://doi.org/10.31362/patd.643460 Y2 - 2020 ER -
EndNote %0 Pamukkale Tıp Dergisi Amyotrofik Lateral Skleroz (ALS) Hastalığının Patogenezi %A Ahmet Doğucem MARANGOZ , Çağdaş ERDOĞAN %T Amyotrofik Lateral Skleroz (ALS) Hastalığının Patogenezi %D 2020 %J Pamukkale Tıp Dergisi %P -1308-0865 %V 13 %N 2 %R doi: 10.31362/patd.643460 %U 10.31362/patd.643460
ISNAD MARANGOZ, Ahmet Doğucem , ERDOĞAN, Çağdaş . "Amyotrofik Lateral Skleroz (ALS) Hastalığının Patogenezi". Pamukkale Tıp Dergisi 13 / 2 (Mayıs 2020): 477-484 . https://doi.org/10.31362/patd.643460
AMA MARANGOZ A , ERDOĞAN Ç . Amyotrofik Lateral Skleroz (ALS) Hastalığının Patogenezi. Pamukkale Tıp Dergisi. 2020; 13(2): 477-484.
Vancouver MARANGOZ A , ERDOĞAN Ç . Amyotrofik Lateral Skleroz (ALS) Hastalığının Patogenezi. Pamukkale Tıp Dergisi. 2020; 13(2): 484-477.