パーキンソン カンレン シッカン ニオケル ケイズガイ チョウオンパ ケンサ ニヨル チュウノウ コクシツ ノ コウキド ヘンカ ノ ケントウ

目 的:パーキンソン病 (Parkinson disease:PD),多系統萎縮症 (multiple system atrophy:MSA),進行性核上性麻痺 (progressive supranuclear palsy:PSP) の患者において経頭蓋超音波検査 (transcranial sonography:TCS) よる中脳黒質の高輝度変化を検討した.方 法:パーキンソン関連疾患連続110 例 (PD 86 例,MSA 12 例,PSP 12 例) と健常者34 例に対しTCSを施行した.中脳黒質を観察しえたPD 47 例,MSA 10 例,PSP 6 例,健常者32 例を解析対象として中脳黒質高輝度所見を評価した.定性評価は高輝度の程度によって視察的にI:none or faint,II:equivocal,III:definite,IV:marked の4 段階に分類した.定量評価は中脳黒質で高輝度変化の面積が0.20 cm2 以上のとき,病的な黒質高輝度変化と定義した.結 果:定性評価では,高輝度範囲が視察的に病的と判定されるIII+IVの割合は,PD 72.4%,MSA 10.0%,PSP 66.7%,健常者3.1%であった.定量評価では,PD 63.8%,MSA 20.0%,PSP 66.7%,健常者9.4%で病的な高輝度変化をみとめた.PD,PSP で病的な高輝度変化の割合が多かった.PSP をPSP-parkinsonism( PSPP)とRichardson\u27s syndrome の2 群に分けた場合,前者では病的な高輝度変化を3 例中3 例 (100%), 後者では3 例中1 例( 33.3%) に認められ,PSP-P で割合が高かった.MSA では10 例中2 例( 20%) に病的な高輝度を認め,いずれもパーキンソン病型の多系統萎縮症であった.結 論:パーキンソン関連疾患における病的な中脳黒質高輝度変化は,疾患特異性というよりも,パーキンソニズムの症候と関連し,ドパミン神経細胞の脆弱性を示す所見と推察された.Objective:We investigated substantia nigra (SN) hyperechogenicitydetermined by transcranial sonography(TCS) to detect abnormalities, and compare findings withthose from Parkinson disease (PD), multiple system atrophy(MSA), progressive supranuclear palsy (PSP) or controlsubjects.Method:In this study, echogenicity of SN was examinedin consecutive 110 parkisonian disorders patients with PD86, MSA12, PSP 11, and 34 control subjects. A sufficientbone window for TCS was available in 47 of 86( 71.2%) inthe PD group, 10 of 12( 86.3 %) in the MSA group, 6 of 11(54.5%) in the PSP group and 32 of 34( 94.1%) in the controlgroup. SN hyperechogenicity was scored using a fourpointscale as follows:I=none or faint, II=equivocal, III=definite, IV=marked. In accordance with previously reportedcut-off values, areas of echogenicity £ 0.19 cm2 wereclassified as normal and areas of echogenicity £ 0.20 cm2were classified as pathological SN hyperechogenicity.Results:The frequency of SN hyperechogenicity, assessedas III and IV scales, was significantly increased in PDpatients, and observed in 72 . 4 % of assessable SN(34/47);qui-squire;p=0.001, vs. controls). The meansize of the SN hyperechogenic area in the PD group, MSAgroup and PSP group was 0.26 cm2±0.13, 0.11 cm2±0.11and 0.23 cm2±0.04, respectively, compared with 0.07 cm2±0.06 in the control group.We have identified two clinical phenotypes, such as Richardson\u27ssyndrome (RS) and PSP-parkinsonism (PSP-P).All of three PSP-P (100%) patients showed a pathologicalSN hyperechogenicity.Conclusion:SN hyperechogenicity was associated with asymptom of parkinsonism rather than disease specificity,and suggested a vulnerability marker of the dopaminergicneuron

Molecular Analysis of the Gene Encoding a Novel Chitin-Binding Protease from Alteromonas sp. Strain O-7 and Its Role in the Chitinolytic System

Alteromonas sp. strain O-7 secretes several proteins in response to chitin induction. We have found that one of these proteins, designated AprIV, is a novel chitin-binding protease involved in chitinolytic activity. The gene encoding AprIV (aprIV) was cloned in Escherichia coli. DNA sequencing analysis revealed that the open reading frame of aprIV encoded a protein of 547 amino acids with a calculated molecular mass of 57,104 Da. AprIV is a modular enzyme consisting of five domains: the signal sequence, the N-terminal proregion, the family A subtilase region, the polycystic kidney disease domain (PkdD), and the chitin-binding domain type 3 (ChtBD3). Expression plasmids coding for PkdD or both PkdD and ChtBD (PkdD-ChtBD) were constructed. The PkdD-ChtBD but not PkdD exhibited strong binding to α-chitin and β-chitin. Western and Northern analyses demonstrated that aprIV was induced in the presence of N-acetylglucosamine, N-acetylchitobiose, or chitin. Native AprIV was purified to homogeneity from Alteromonas sp. strain O-7 and characterized. The molecular mass of mature AprIV was estimated to be 44 kDa by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. The optimum pH and temperature of AprIV were pH 11.5 and 35°C, respectively, and even at 10°C the enzyme showed 25% of the maximum activity. Pretreatment of native chitin with AprIV significantly promoted chitinase activity

Outcomes in Newly Diagnosed Atrial Fibrillation and History of Acute Coronary Syndromes: Insights from GARFIELD-AF

BACKGROUND: Many patients with atrial fibrillation have concomitant coronary artery disease with or without acute coronary syndromes and are in need of additional antithrombotic therapy. There are few data on the long-term clinical outcome of atrial fibrillation patients with a history of acute coronary syndrome. This is a 2-year study of atrial fibrillation patients with or without a history of acute coronary syndromes