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首页> 外文期刊>Cardiovascular pathology: the official journal of the Society for Cardiovascular Pathology >Progressive heart disease in mucopolysaccharidosis type I mice may be mediated by increased cathepsin B activity
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Progressive heart disease in mucopolysaccharidosis type I mice may be mediated by increased cathepsin B activity

机译：粘性多族种中的渐进心脏病I型小鼠可以通过增加的组织蛋白酶B活性介导

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Mucopolysaccharidosis type I (MPS I) is a lysosomal disorder characterized by a deficiency of alpha-L-iduronidase and storage of undegraded glycosaminoglycans (GAGs). Clinical findings of the disease include heart failure, and patients often need valve replacement. It has been shown that, later in life, MPS I mice develop those abnormalities, but to date, there have not been studies on the progression and pathogenesis of the disease. Therefore, in the present study, we evaluated heart function in normal and MPS I male mice from 2 to 8 months of age. Echocardiographic analysis showed left ventricular enlargement with progressive reduction in ejection fraction, fractional area change, and left ventricular fractional shortening in the MPS I hearts at 6 and 8 months of age and a reduction in acceleration time/ejection time ratio of the pulmonary artery starting at 6 months of age, which suggests pulmonary vascular resistance. Histological and biochemical analysis confirmed progressive GAG storage from 2 months of age and onwards in the myocardium and heart valves, which had also increased in thickness. Additionally, macrophages were present in the MPS I heart tissue. Collagen content was reduced in the MPS I mouse valves. Cathepsin B, an enzyme that is known to be able to degrade collagen and is involved in heart dilatation, displayed a marked elevation in activity in the MPS I mice and could be responsible for the heart dilatation and valves alterations observed. Our results suggest that the MPS I mice have progressive heart failure and valve disease, which may be caused by cathepsin B overexpression. (C) 2017 Elsevier Inc. All rights reserved.
机译：粘性多族种I型（MPS I）是一种溶酶体紊乱，其特征，其特征在于α-L-致抗糖苷酶的缺乏，并储存未扩张的糖胺聚糖（GAG）。疾病的临床发现包括心力衰竭，患者通常需要瓣膜置换。已经表明，稍后在生命中，MPS I小鼠培养了这些异常，但到目前为止，还没有研究疾病的进展和发病机制。因此，在本研究中，我们评估了正常和MPS I雄性小鼠的心脏功能，从2至8个月的年龄。超声心动图分析显示左心室扩大射血分数，分数区域变化和MPS I心中的左心室分数缩短，在6和8个月的时间内，肺动脉的加速时间/喷射时间比的降低6个月的年龄，表明肺血管阻力。组织学和生化分析证实了从2个月的年龄和心肌和心脏瓣膜上的渐进式Gag储存，厚度增加。另外，巨噬细胞存在于MPS I心脏组织中。在MPS I鼠标阀中降低了​​胶原蛋白含量。 Compapsin B，已知能够降解胶原蛋白并参与心脏扩张的酶，在MPS I小鼠中显示出明显的升高，并且可以负责观察心脏扩张和阀门的变化。我们的研究结果表明，MPS I小鼠具有渐进心力衰竭和瓣膜疾病，其可能是由组织蛋白酶B过表达引起的。 （c）2017年Elsevier Inc.保留所有权利。

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