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    Conformational changes of wild-type SOD1 affect the dimerization at ambient temperature
    (Koç University, 2022) Yıldırım, Günseli; Demirci, Hasan; 0000-0002-9135-5397; Koç University Graduate School of Sciences and Engineering; Molecular Biology and Genetics; 307350
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    Investigating the synaptic physiology of renshaw cells and their influence on motoneuron degeneration
    (Koç University, 2019) Özyurt, Mustafa Görkem; Türker, Kemal Sıtkı; 0000-0001-9962-075X; Koç University Graduate School of Sciences and Engineering; Bio-Medical Sciences and Engineering; 6741
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    Investigation of the biochemical parameters in pre-symptomatic and symptomatic SOD1 mutated rat model of ALS
    (Koç University, 2021) Aydemir, Duygu; Ulusu, Nuriye Nuray; 0000-0002-3173-1389; Koç University Graduate School of Health Sciences; Cellular and Molecular Medicine; 6807
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    Stereological and electrophysiological evaluation of autonomic involvement in amyotrophic lateral sclerosis
    (Elsevier, 2022) Öztürk, Rüstem; Karlsson, Pall; Hu, Xiaoli; Akdeniz, Esra; İşak, Barış; Sürücü, Hüseyin Selçuk; Faculty Member; School of Medicine; 21780
    Objective: Previous studies have identified autonomic dysfunction in amyotrophic lateral sclerosis (ALS) using mostly neurophysiological techniques. In this study, stereological evaluation of autonomic fibers and sweat glands has been performed to identify structural evidence of autonomic denervation in patients with ALS. Methods: In this study, 29 ALS patients were compared to 29 controls using COMPASS-31 questionnaire, sympathetic skin response (SSR), and heart rate variability (HRV) at rest. From the same cohorts, 20 ALS patients and 15 controls were further evaluated using staining of autonomic nerve fibers and sweat glands in skin biopsies. SSR and resting HRV were repeated in the ALS patient cohort one year later. Results: COMPASS-31 total score, gastrointestinal- and urinary-sub scores were higher in ALS patients than controls (P = 0.004, P = 0.005, and P = 0.049, respectively). In the ALS patient cohort, SSR amplitudes in hands and feet were lower than in controls (P<0.0001 and P = 0.0009, respectively), but there was no difference in resting HRV (P>0.05). While there was no change in nerve fibers innervating sweat glands, their density was lower in ALS patients than controls, and semi-quantitative analysis also showed structural damage (P = 0.02 and P = 0.001, respectively). SSR and resting HRV of ALS patients remained stable during the one-year follow-up period (P>0.05). Discussion: Supporting abnormal neurophysiological tests, stereological analysis revealed direct evidence of autonomic denervation in ALS patients. However, the degenerative process in autonomic nerve fibers is relatively slow, compared to the rate of motor neuron degeneration in this condition.