NEUROCHEMISTRY OF THE MUCOPOLYSACCHARIDOSES: BRAIN LIPIDS AND LYSOSOMAL ENZYMES IN PATIENTS WITH FOUR TYPES OF MUCOPOLYSACCHARIDOSIS AND IN NORMAL CONTROLS
NEUROCHEMISTRY OF THE MUCOPOLYSACCHARIDOSES: BRAIN LIPIDS AND LYSOSOMAL ENZYMES IN PATIENTS WITH FOUR TYPES OF MUCOPOLYSACCHARIDOSIS AND IN NORMAL CONTROLS
复制标题
粘多糖症的神经化学：四种类型粘多糖症患者和正常对照者的脑脂质和溶酶体酶
作者：
G. Constantopoulos;A. Dekaban
摘要：在未受影响的对照组和六名粘多糖贮积症（MPS）患者的大脑灰质和白质以及肝脏中测量了脂质和某些溶酶体酶。其中三名患者患有 I 型 MPS (Hurler)、一名 II 型 (Hunter)、一名 IIIA 型 (Sanfilippo A) 和一名 V 型 (Scheie)。这些组织的糖胺聚糖 (GAG) 先前已得到充分表征（Constantopouloset al., 1976）。
Abstract— Lipids and certain lysosomal enzymes were measured in the cerebral gray and white matter and in the liver of unaffected controls and six patients with mucopolysaccharidosis (MPS). Three of the patients had MPS Type I (Hurler), one Type II (Hunter), one Type IIIA (Sanfilippo A) and one Type V (Scheie). The glycosaminoglycans (GAG) of those tissues have been fully characterized previously (Constantopouloset al., 1976).