James H. Austin

A CONTROLLED STUDY OF ENZYMIC ACTIVITIES IN THREE HUMAN DISORDERS OF GLYCOLIPID METABOLISM.

A CONTROLLED STUDY OF ENZYMIC ACTIVlTIES IN THREE HUMAN DISORDERS OF GLYCOLIPID METABOLISM” JAMES H. AUSTIN Division of Neurology, University of Oregon Medical School, Portland, Oregon, U.S.A. A . s. BALASUBRAMANIAN~, T. N. PATTABIRAMAN

Metachromatic Sulfatides in Cerebral White Matter and Kidney.

, s. SARASWATHI, D. K. BASUS and B. K. BACHHAWAT Neurochemistry Laboratory, Department of Neurology and Neurosurgery, Christian Medical College & Hospital, Vellore, S. India

A new spectrophotometric method for the micro determination of sulfuric acid esters in sulfatide fractions

Summary 1) Metachromatic sulfuric acid esters (SAE) were measured in sulfatide fractions extracted from normal and abnormal white matter and kidney. 2) Slightly lower than average SAE values for white matter were found in early childhood, in old age, and in diseases interfering with myelin metabolism. 3) The average SAE value in normal mature white matter was 58 mg/g. This was 10 times the value for normal kidney and corresponds with the much greater lipid metachromasia in white matter histologically. 4) Statistically significant elevations in sulfatide values were found in white matter and kidney in one disease (metachromatic leucoencephalopathy). 5) This accumulation may be related to an error in sulfatide metabolism, and appears, descriptively, to constitute a “sulfatide lipidosis.”

Significance of plasma glycolipid levels in normals and in 3 disorders of brain glycolipids.

SummaryA new micro method has been developed for measuring sulfatide fractions. Sulfuric acid ester absorption is measured at 8.02 μm (μ) by infrared spectrophotometry, compared with that of a standard sulfatide, and corrected for phosphorus. General problems in measuring sulfatides are noted; procedural details, control studies, advantages, and limitations of the present method are discussed.ZusammenfassungEine neue Mikromethode für die Bestimmung von Sulfatidfraktionen wird beschrieben. Die Absorption der Schwefelsäureester wird im Infrarot bei 8,02 μm gemessen, mit jener eines Sulfatidstandards verglichen und für Phosphor korrigiert. Allgemeine, mit der Bestimmung von Sulfatiden verbundene Probleme werden erwähnt; technische Einzelheiten, Kontroll-untersuchungen, Vorteile und Grenzen der beschriebenen Methode werden besprochen.RésuméMise au point dune nouvelle microméthode pour la détermination de fractions de sulfatides. Labsorption de lester sulfurique est mesurée à 8,02 μm (μ) par spectrophotométrie infra-rouge et comparée avec celle dun sulfate standard puis corrigée du fait de la présence du phosphore. Les auteurs rappellent les problèmes généraux rencontrés dans la détermination des sulfatides. Les détails opératoires, les études de contrôle, les avantages et les limitations de la présente méthode sont dautre part discutés.

Studies in Metachromatic Leukodystrophy: XII. Multiple Sulfatase Deficiency

Summary 1. Plasma glycolipids and other plasma lipids were studied in 2 diseases affecting the myelin glycolipids, in gargoylism, and in controls of appropriate age. 2. Plasma glycolipid levels in this series of normal children ranged from 4.9-6.5 mg%. Those in 2 metachromatic children ranged from 6.0-6.3 mg%. In the globoid child. plasma glycolipids ranged from 4.3-5.1 mg%. 3. Data obtained with these methods would not appear to establish a disorder of total plasma glycolipids per se as the primary source for systemic deposits in the patients studied. 4) The significance of the plasma glycolipid level is considered, and the need for individualization in further studies is stressed. It is a pleasure to acknowledge the assistance of the Dept. of Pediatrics and of Mrs. Margaret Bischel in obtaining plasma samples, and that of Mrs. Jean Scott and Mr. David Gaudin in cholesterol and phosphorous determinations.