Artikel
Cerebrospinal fluid (CSF) : Transthyretin as protein marker for Moyamoya disease
Liquor cerebrospinals (CSF): Transthyretin als Proteinmarker bei Moyamoya Erkrankung
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Autoren
Veröffentlicht: | 4. Mai 2005 |
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Gliederung
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Objective
Moyamoya disease (MMD) is a rare entity that results in progressive occlusion of the arteries of the circle of Willis. Although the cause of MMD remains undetermined, evidence supports infectious and auto-immun relateded origin, suggesting humorally mediated initiation of MMD. In order to further elucidate these findings, proteome analysis of cerebrospinal fluid (CSF) was performed in patients with MMD.
Methods
Thirty patients (22 adults [19 female, 3 male]; 8 children [5 female, 3 male]) with confirmed MMD and 10 patients (2 female, 8 male) with atherosclerotic steno-occlusive disease who served as controls were enrolled in the present study. Pooled and individual CSF samples were analyzed using a 2-D gel-electrophoresis approach. Abundant proteins, albumin and immunoglobulines, were removed prior to ioselectric focusing. Fluorescent gel stain was used to do a differential proteome analysis with concurrent in-gel protein quantitation.
Results
Ten differentially expressed proteins were excised followed by in-gel digestion and peptide sequencing by tandem mass spectrometry (MS). One Protein, a mutated Transthyretin, was present in 71% of all MMD patients regardless their sex and age and in 80% of the adult patients, whereas it was not found in control persons. The performed Nano ESI (Electrospray-imaging)- MS/ MS sequence analysis of the Transthyretin revealed a specific post-translational modification of glutaric acid at postion 61.
Conclusions
Transthyretin can be used as a specific, but not quantitative disease marker for MMD.