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Influence of aging on the protein profile of myelin isolated from human brain white matter

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Niebroj-Dobosz I., Rafalowska J., Lukasiuk M., Wisniewska W., Dziewulska D.
Neuropatologia Polska
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1992
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vol. 30
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issue 2
147-154
EN
Myelin proteins composition was examined in material of 20 autoptic cases at ages from 20 to 97 years. The technique of polyacrylamide gel electrophoresis and isotachophoresis was applied. In polyacrylamide gel electrophoresis a progressive increase starting at the age of 60 years of Wolfgram protein at the expense of Folch-Less proteolipid protein and DM-20 protein was observed. The myelin-associated protein started to increase in the 4th and 5th decade of life, returning therafter to values observed in younger cases. The isotachophoretic technique did not differentiate the changes observed in myelin protein in the course of aging.
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Matrix metalloproteinases in serum of Emery-Dreifuss muscular dystrophy patients

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Niebroj-Dobosz I., Madej-Pilarczyk A., Marchel M., Sokołowska B., Hausmanowa-Petrusewicz I.
Acta Biochimica Polonica
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2009
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vol. 56
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issue 4
717-722
EN
In the pathogenesis of dilated cardiomyopathy (DCM) in Emery-Dreifuss muscular dystrophy (EDMD) matrix metalloproteinases (MMPs) are supposed to be involved and may have diagnostic/prognostic value. Serum levels of MT1-MMP, MMP-2 and MMP-9 were quantified by ELISA and zymography in 22 EDMD patients and 15 age-matched controls. In the autosomal-dominant EDMD MMP-2 and MT1-MMP were increased in all cases, and MMP-9 was increased in two of the eight examined patients. In the X-linked EDMD MMP-2 expression was increased in all the cases, MMP-9 level was elevated in 3 of the 14 cases, and MT1-MMP was decreased in eight of these patients. There was no evident correlation between the MMPs level and the different cardiac parameters including left-ventricular end-diastolic diameter, left atrial diameter and left ventricular ejection fraction in either form of EDMD. The presented results indicate that a changed level of matrix metalloproteinases, especially that of MMP-2 in serum, may be of value for detection of cardiac involvement in EDMD patients, especially in those patients with no evident subjective cardiac symptoms. Further follow-up studies of MMPs are needed to check if their determination is of value for monitoring of the progression of atrial/ventricular dilatation. MMPs determinations may also be useful for monitoring DCM treatment by synthetic MMPs inhibitors.
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