PMID- 19796283 OWN - NLM STAT- MEDLINE DCOM- 20100817 LR - 20151113 IS - 1468-1331 (Electronic) IS - 1351-5101 (Linking) VI - 17 IP - 2 DP - 2010 Feb TI - Matrix metalloproteinases and their tissue inhibitors in serum and cerebrospinal fluid of patients with amyotrophic lateral sclerosis. PG - 226-31 LID - 10.1111/j.1468-1331.2009.02775.x [doi] AB - BACKGROUND AND PURPOSE: Matrix metalloproteinases (MMPs) are implicated in the pathogenesis of motor neuron degeneration in amyotrophic lateral sclerosis (ALS). We investigated the expression of MMPs and tissue inhibitors of matrix metalloproteinases (TIMPs) in serum and cerebrospinal fluid (CSF) correlating the results with age, disease duration and the clinical course. METHODS: The material consisted of 30 ALS patients and 15 age-matched healthy controls. ELISA method to determine the expression of MT-MMP-1, MMP-2, MMP-9, TIMP-1 and TIMP-2 in serum and CSF was used. MMP-2 and MMP-9 by zymography was also tested. RESULTS: In serum MT-MMP-1, MMP-2, MMP-9 and TIMP-1 expression was increased, especially in mild ALS cases. TIMP-2 values were normal. In CSF MT-MMP-1, MMP-2 and TIMP-1 level was either increased or normal, that of MMP-9 was decreased. TIMP-2 did not change. No correlation of MMPs and TIMP-1 expression in serum and CSF and the age of the patients was found. A correlation was observed between MMPs and TIMPs and disease duration. CONCLUSIONS: Increased level of MMPs and TIMP-1 of ALS patients may reflect the degeneration process of motor neurons and skeletal muscles and/or is associated with tissues remodeling. The low level of MMP-9 in CSF may result from impaired balance between MMP-9 and TIMP-1 and/or its increased intrathecal degradation and physical clearance. Although the role of changed MMPs/TIMPs level in the pathogenesis of ALS is not clear their analysis in serum may be used as prognostic factor and a potential marker for monitoring treatment effects. FAU - Niebroj-Dobosz, I AU - Niebroj-Dobosz I AD - Neuromuscular Unit, Medical Research Center, Polish Academy of Sciences, Warsaw, Poland. dobosz@cmdik.pan.pl FAU - Janik, P AU - Janik P FAU - Sokolowska, B AU - Sokolowska B FAU - Kwiecinski, H AU - Kwiecinski H LA - eng PT - Journal Article DEP - 20091001 PL - England TA - Eur J Neurol JT - European journal of neurology JID - 9506311 RN - 0 (Tissue Inhibitor of Metalloproteinase-1) RN - 0 (Tissue Inhibitor of Metalloproteinases) RN - 127497-59-0 (Tissue Inhibitor of Metalloproteinase-2) RN - EC 3.4.24.- (Matrix Metalloproteinases) RN - EC 3.4.24.24 (MMP2 protein, human) RN - EC 3.4.24.24 (Matrix Metalloproteinase 2) RN - EC 3.4.24.35 (Matrix Metalloproteinase 9) RN - EC 3.4.24.7 (MMP1 protein, human) RN - EC 3.4.24.7 (Matrix Metalloproteinase 1) SB - IM MH - Adult MH - Aged MH - Amyotrophic Lateral Sclerosis/*blood/*cerebrospinal fluid MH - Case-Control Studies MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Male MH - Matrix Metalloproteinase 1/blood/cerebrospinal fluid MH - Matrix Metalloproteinase 2/blood/cerebrospinal fluid MH - Matrix Metalloproteinase 9/blood/cerebrospinal fluid MH - Matrix Metalloproteinases/*blood/*cerebrospinal fluid MH - Middle Aged MH - Severity of Illness Index MH - Tissue Inhibitor of Metalloproteinase-1/blood/cerebrospinal fluid MH - Tissue Inhibitor of Metalloproteinase-2/blood/cerebrospinal fluid MH - Tissue Inhibitor of Metalloproteinases/*blood/*cerebrospinal fluid EDAT- 2009/10/03 06:00 MHDA- 2010/08/18 06:00 CRDT- 2009/10/03 06:00 PHST- 2009/10/03 06:00 [entrez] PHST- 2009/10/03 06:00 [pubmed] PHST- 2010/08/18 06:00 [medline] AID - ENE2775 [pii] AID - 10.1111/j.1468-1331.2009.02775.x [doi] PST - ppublish SO - Eur J Neurol. 2010 Feb;17(2):226-31. doi: 10.1111/j.1468-1331.2009.02775.x. Epub 2009 Oct 1.