抄録
Abnomal substances accumulated in the liver of a patient died of a familial metabolic disorder were isolated. They are complexes of protein and heteropolysaccharide, composed of a large amount of glucose and a smaller amount of glucosamine and galactosamine, showing a blue iodine reaction. The iodine reaction of the native complex was diminished by digestion with peptide hydrolase and one of the fraction obtained after digestion was resistant to digestion with α-amylase.
This investigation was initiated by the proposal of Dr. T. Sano, formerly Prof. of Pediatrics, Tohoku University, and the work was continued by the kind courtesy and interest of Dr. T. Arakawa, Prof. of Pediatrics, Tohoku University, who allowed one of the authors (N. K.) to carry out cooperative experiments in the Department of Biochemistry, Tohoku Pharmaceutical School. The authors are also grateful to Dr. K. Akazaki, Prof. of Pathology, Tohoku University, for his interest and encouragement.
A part of this investigation was supported by a Grant-in-Aid for Scientific Research, Ministry of Education, which is gratefully acknowledged.