Trihexoside

Greener JE, Poorthuis BJ, Kuijper S, Helmond MTJ, Hollak CE, Aerts JM (2007) HPLC for simultaneous quantification of total ceramide, glucosylceramide, and ceramide trihexoside concentrations in plasma. Clin Chem 53 742-747... 

These same human sera and some of the rabbit sera also were tested against ceramide trihexoside (CTH) and digalactosyl diglyceride (Table I). Several of the human sera reacted with digalactosyl diglyceride, in confirmation of the results of Hirsch and Parks ( 5). Although only a few human sera reacted with CTH, all of the rabbit sera tested showed reactivity. 

TABLE I. HUMAN AND RABBIT NATURAL ANTIBODIES AGAINST DIGALACTOSYL DIGLYCERIDE AND CERAMIDE TRIHEXOSIDE... 

In addition to the cerebrosides, which contain only one carbohydrate residue, there are other glycosphingolipids in mammalian cells that contain more than one sugar component. These oligosaccharide derivatives are called globosides. For example, lactosyl ceramide (1-O-lactosyl-A acylsphingosine) is a constituent of the erythrocyte membrane. Ceramide trihexoside accumulates in the kidneys of patients with Fabry s disease, due to the lack of a lysosomal a-galactosidase A activity. 

Gm2 gangliosidosis is caused by a deficiency of hexosamidase A and B. It was described for the first time by K.. Sandhoff et al. as Sandhoffs disease in 1968. This biochemical variant (type II) largely corresponds to Tay-Sachs disease, which is also autosomal recessive. Renal globoside (ceramide trihexoside) is stored in the visceral organs, particularly in the liver and spleen. Hepatomegaly is present, occasionally with splenomegaly. The lysosomes within the hepatocytes become considerably larger until they are as big as the nucleus and show lamellar structures. 

Mills K., Johnson A., Winchester B., Synthesis of novel internal standards for the quantitative determination of plasma ceramide trihexoside in Fabry disease by tandem mass spectrometry, FEBS letters 515 (2002) 171-176. 

Scheme 5. Biosynthesis of Glycosphingolipids. (For the structure of various sphingolipids, see the review by Kiss. Structure of ceramide trihexoside and globoside is based on Refs. 428 and 429, respectively.)...

Fabry s disease Reddish-purple skin rash, kidey failure, pain in lower extremities Ceramide trihexoside Ceramide trihexoside -galactosidase... 

Ceramide trihexosidase (a-galactosidase) (EC 3.2.1.22). Sphingolipidosis due to accumulation of ceramide trihexoside. Skin lesions (purple macules and papules). Corneal opacities, cataracts, retinal edema. Cardiovascular, neurological and gastrointestinal disorders. Characteristic severe burning pains in extremities. X-chromosome-linked. 

The terminal disaccharide of the ceramide trihexoside isol. from normal human kidney and erythrocytes a dcgradn. prod, of mucilage from common okra (Hibiscus esculentus) formed by the action of P-galactosidase on i>galactose The simplest structural unit capable of bacterial fimbriae binding associated with initiation of bacterial infections. Cryst. (MeOH/ l-butanol/H20). 

Cis- and trans-sinapine (the choline ester of sinapic acid), sinapic acid, sinapoyl glucose, Kaempferol-sinapoyl-trihexoside 1 -0-P-D-glucopyranosyl sinapate, Sinapoyl-hexoside, Disinapoyl hexoside, trisinapoyl-dihexoside and sinapoyl conjugate... 

Human plasma a-D-galactosidase A has been purified 7000 fold from Cohn Fraction IV-I on a pilot scale. The ratio of activities against 4-methylumbelliferyl a-D-galactopyranoside and ceramide trihexoside was 6.2 1 both activities were heat labile and exhibited the same mobilities on polyacrylamide gel electrophoresis. 

The ceramide trihexoside (6) accumulating in Fabry s disease has been synthesized by a conventional Koenigs-Knorr procedure. A novel ganglioside, disialosylparagloboside (7), has been isolated from human kidneys and its structure determined. ... 

This ceramide trihexoside has also been isolated from normal kidney (Makita et al. 1964). Other ceramide trihexosides, which are present in liver, spleen and serum (Svennerholm et al. 1963), contain, in addition to the ceramide lactoside, galactose or N-acetyl-galactosamine, linked to C-3 or C-4 of the terminal galactose ... 

Biochemistry of organ glycolipids. III. The structures of human kidney cerebroside sulfuric ester, ceramide dihexoside and ceramide trihexoside. J. Biochem. (Tokyo) 66,365 (1964). 

SwEELEY and Klionsky (1963, 1964, 1966) isolated from organs of ACD patients two glycolipids, which they identified as ceramide-dihexoside and ceramide-trihexoside. Their findings confirmed the assumption of Ruiter et al. (1947) who suggested classification of ACD as a lipid storage disease. They did not confirm the postulate that the stored material was a phospholipid. 

The fatty acids of the trihexoside consist to 75% of C22—C24 fatty acids while palmitic and stearic acids together amount to only about 10%. 

It is probable that the stored lipid of ACD occurs normally in small amounts. SvENNERHOLM and SvENNERHOLM (1963) found a trihexoside with a proportion of ceramide glucose galactose of 1 1 2 in plasma and Martens son et al. (1966) found a trihexoside in kidney lipids of normals in addition to dihexoside. 

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