Creatine Kinase and Progression Rate in Amyotrophic Lateral Sclerosis.

Autor: Ceccanti M; Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy., Pozzilli V; Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy., Cambieri C; Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy., Libonati L; Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy., Onesti E; Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy., Frasca V; Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy., Fiorini I; Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy., Petrucci A; Centre for Neuromuscular and Neurological Rare Diseases, San Camillo Forlanini Hospital, 00152 Rome, Italy., Garibaldi M; Neuromuscular Disease Centre, Department of Neurology, Mental Health and Sensory Organs (NESMOS), Sant'Andrea Hospital, Sapienza University of Rome, 00189 Rome, Italy., Palma E; Department of Physiology and Pharmacology, Laboratory Affiliated to Istituto Pasteur Italia, Sapienza University of Rome, 00185 Rome, Italy., Bendotti C; Laboratory Molecular Neurobiology, Department of Neuroscience, Istituto di Ricerche Famacologiche Mario Negri-IRCCS, 20156 Milan, Italy., Fabbrizio P; Laboratory Molecular Neurobiology, Department of Neuroscience, Istituto di Ricerche Famacologiche Mario Negri-IRCCS, 20156 Milan, Italy., Trolese MC; Laboratory Molecular Neurobiology, Department of Neuroscience, Istituto di Ricerche Famacologiche Mario Negri-IRCCS, 20156 Milan, Italy., Nardo G; Laboratory Molecular Neurobiology, Department of Neuroscience, Istituto di Ricerche Famacologiche Mario Negri-IRCCS, 20156 Milan, Italy., Inghilleri M; Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy.
Jazyk: angličtina
Zdroj: Cells [Cells] 2020 May 08; Vol. 9 (5). Date of Electronic Publication: 2020 May 08.
DOI: 10.3390/cells9051174
Abstrakt: Amyotrophic lateral sclerosis ( ALS) is a neurodegenerative disease with no recognized clinical prognostic factor. Creatinine kinase (CK) increase in these patients is already described with conflicting results on prognosis and survival. In 126 ALS patients who were fast or slow disease progressors, CK levels were assayed for 16 months every 4 months in an observational case-control cohort study with prospective data collection conducted in Italy. CK was also measured at baseline in 88 CIDP patients with secondary axonal damage and in two mouse strains (129SvHSD and C57-BL) carrying the same SOD1G93A transgene expression but showing a fast (129Sv-SOD1G93A) and slow (C57-SOD1G93A) ALS progression rate. Higher CK was found in ALS slow progressors compared to fast progressors in T1, T2, T3, and T4, with a correlation with Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) scores. Higher CK was found in spinal compared to bulbar-onset patients. Transgenic and non-transgenic C57BL mice showed higher CK levels compared to 129SvHSD strain. At baseline mean CK was higher in ALS compared to CIDP. CK can predict the disease progression, with slow progressors associated with higher levels and fast progressors to lower levels, in both ALS patients and mice. CK is higher in ALS patients compared to patients with CIDP with secondary axonal damage; the higher levels of CK in slow progressors patients, but also in C57BL transgenic and non-transgenic mice designs CK as a predisposing factor for disease rate progression.
Competing Interests: The authors declare no conflict of interest. The funder had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.
Databáze: MEDLINE
Externí odkaz:
Nepřihlášeným uživatelům se plný text nezobrazuje