Investigations of Microtubule-associated Protein 2 Gene Expression in Spinal Muscular Atrophy


BORA G. , SUCULARLI C. , Hensel N., Claus P., Yurter H. E.

JOURNAL OF PEDIATRIC RESEARCH, cilt.6, ss.148-154, 2019 (ESCI İndekslerine Giren Dergi) identifier

  • Cilt numarası: 6 Konu: 2
  • Basım Tarihi: 2019
  • Doi Numarası: 10.4274/jpr.galenos.2019.71473
  • Dergi Adı: JOURNAL OF PEDIATRIC RESEARCH
  • Sayfa Sayıları: ss.148-154

Özet

Aim: Spinal muscular atrophy (SMA) is a devastating genetic disease in childhood andff is caused by the absence of functional survival motor neuron (SMN) protein, which leads to impairments of the cytoskeleton, especially in neurons. Dysregulation of actin dynamics have been linked to SMA patho mechanisms, however involvement of altered microtubule dynamics is largely unknown. In this study, we investigated differentially expressed microtubule-related genes using in vitro and in vivo SMA model systems.