Next Article in Journal
The Expression of microRNA in Adult Rat Heart with Isoproterenol-Induced Cardiac Hypertrophy
Next Article in Special Issue
Modulation of Inflammasome and Pyroptosis by Olaparib, a PARP-1 Inhibitor, in the R6/2 Mouse Model of Huntington’s Disease
Previous Article in Journal
Membrane Binding Promotes Annexin A2 Oligomerization
Open AccessArticle

Creatine Kinase and Progression Rate in Amyotrophic Lateral Sclerosis

1
Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy
2
Centre for Neuromuscular and Neurological Rare Diseases, San Camillo Forlanini Hospital, 00152 Rome, Italy
3
Neuromuscular Disease Centre, Department of Neurology, Mental Health and Sensory Organs (NESMOS), Sant’Andrea Hospital, Sapienza University of Rome, 00189 Rome, Italy
4
Department of Physiology and Pharmacology, Laboratory Affiliated to Istituto Pasteur Italia, Sapienza University of Rome, 00185 Rome, Italy
5
Laboratory Molecular Neurobiology, Department of Neuroscience, Istituto di Ricerche Famacologiche Mario Negri-IRCCS, 20156 Milan, Italy
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
Cells 2020, 9(5), 1174; https://doi.org/10.3390/cells9051174
Received: 3 April 2020 / Revised: 29 April 2020 / Accepted: 6 May 2020 / Published: 8 May 2020
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. View Full-Text
Keywords: prognosis; amyotrophic lateral sclerosis; chronic inflammatory demyelinating polyneuropathy; CK; creatine kinase; neurodegenerative disease prognosis; amyotrophic lateral sclerosis; chronic inflammatory demyelinating polyneuropathy; CK; creatine kinase; neurodegenerative disease
Show Figures

Figure 1

MDPI and ACS Style

Ceccanti, M.; Pozzilli, V.; Cambieri, C.; Libonati, L.; Onesti, E.; Frasca, V.; Fiorini, I.; Petrucci, A.; Garibaldi, M.; Palma, E.; Bendotti, C.; Fabbrizio, P.; Trolese, M.C.; Nardo, G.; Inghilleri, M. Creatine Kinase and Progression Rate in Amyotrophic Lateral Sclerosis. Cells 2020, 9, 1174.

Show more citation formats Show less citations formats
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Search more from Scilit
 
Search
Back to TopTop