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Current trends in sphingolipidoses and allied disorders
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Current Trends in Sphingolipidoses and Allied Disorders ( A d v a n c e s in E x p e r i m e n t a l M e d i c i n e a n d B i o l o g y , Vol. 68) ( P r o c e e d i n g s o f t h e 5th I n t e r n a t i o n a l S y m p o s i u m o n C u r r e n t T r e n d s in S p h i n g o l i p i d o s e s a n d A l l i e d D i s o r d e r s , B r o o k l y n , N . Y . , 20-21 O c t o b e r , 1975), by B. W . V o l k a n d L. S c h n e c k (Eds.), xi + 612 pages, i l l u s t r a t e d , w i t h tables, P l e n u m Press, N e w Y o r k , L o n d o n , 1976, U S $ 54.00. This book presents the papers from a Symposium held in New York in October 1975. Since the last such meeting in 1971, much work has shown that the lipidoses and leucodystrophies are close metabolic cousins. However, the closer linkages with fucosidosis, mannosidosis and Hunter's Syndrome, to name a few, are becoming even more apparent. The rate at which the field continues to move can be assessed from the Conference Summary statement that only 3 of the 16 disease states had unknown enzyme defects at the time of writing. But the recent findings of L. S. Wolfe will probably remove yet another disease (neuronal ceroid lipofuscinosis) from the list of the unknown chemical pathologies. Recent interest has centred upon single cell assays for specific enzymes in order to make a diagnosis early in pregnancy with only a few cells. This has been reached by using small volumes (0.3 #1) and long incubations (10 hr). Since it is now possible to synthesise genes and to have them spell out their phenotype in cells, a high level of interest continues in the role of enzyme replacements. The book is of primary interest to paediatric neurologists, developmental biochemists, neurochemical patholog:sts and those concerned with chemical aspects of mental retardation. It gives a well-balanced sample of the problems relating to genetic defects in cleaving complex carbohydrate linkages in general and, of course, the sphingolipidoses in fine detail. E. J. Thompson
Human Memory - - The Processing of Information, by G . R. L o f t u s a n d E. F. L o f t u s , xii ÷
179 pages, 91 i l l u s t r a t i o n s , 6 table~, L a w r e n c e E r l b a u m A s s o c i a t e s , H i l l d a l e ,
N . J . , d i s t r i b u t e d by J o h n W i l e y a n d Sons, Ltd., C h i c h e s t e r , 1976, £ 6.60. U S $ 12.25. This book surveys attempts at the experimental unravelling of the nature of memory. An informationprocessing theoretical point of view provides a helpful framework within which to organize the data. The questions asked concern the storage, coding, transfer, loss and retrieval of information and key experiments are described in support of the explanations offered. In this way quite an advanced level of treatment is achieved without attempting a comprehensive review. Some active controversial issues are by-passed but would soon be encountered by anyone following up the well chosen references. Several chapters deal in succession with the evidence for and nature of the initial very brief sensory storage of unanalysed information, selective transfer to a limited capacity, less brief, but still short-term store of mainly acoustically coded information, and further transfer, involving organised forms of semantic coding, to a long-term store. Retrieval and loss from these stores are discussed as are the important differences between recall and recognition and between the remembering of events and of stored conceptual knowledge. A final chapter is devoted to practical applications of memory research in education, law, human engineering and clinical assessment. The clinical section is not very impressive and clinical evidence gains scant attention in the main chapters. The neurobiologist will not find in this book, nor in this field of study, a functional analysis of processes which can be closely correlated with his knowledge of brain structure and physiological functioning. He will find a well argued and readable account of phenomena which he will ultimately need to reconcile with his own studies. Psychologists will find an excellent introduction to the study of memory which is likely to become the prescribed text for many academic courses on this topic. H. Gwynne Jones
Current Trends in Sphingolipidoses and Allied Disorders ( A d v a n c e s in E x p e r i m e n t a l M e d i c i n e a n d B i o l o g y , Vol. 68) ( P r o c e e d i n g s o f t h e 5th I n t e r n a t i o n a l S y m p o s i u m o n C u r r e n t T r e n d s in S p h i n g o l i p i d o s e s a n d A l l i e d D i s o r d e r s , B r o o k l y n , N . Y . , 20-21 O c t o b e r , 1975), by B. W . V o l k a n d L. S c h n e c k (Eds.), xi + 612 pages, i l l u s t r a t e d , w i t h tables, P l e n u m Press, N e w Y o r k , L o n d o n , 1976, U S $ 54.00. This book presents the papers from a Symposium held in New York in October 1975. Since the last such meeting in 1971, much work has shown that the lipidoses and leucodystrophies are close metabolic cousins. However, the closer linkages with fucosidosis, mannosidosis and Hunter's Syndrome, to name a few, are becoming even more apparent. The rate at which the field continues to move can be assessed from the Conference Summary statement that only 3 of the 16 disease states had unknown enzyme defects at the time of writing. But the recent findings of L. S. Wolfe will probably remove yet another disease (neuronal ceroid lipofuscinosis) from the list of the unknown chemical pathologies. Recent interest has centred upon single cell assays for specific enzymes in order to make a diagnosis early in pregnancy with only a few cells. This has been reached by using small volumes (0.3 #1) and long incubations (10 hr). Since it is now possible to synthesise genes and to have them spell out their phenotype in cells, a high level of interest continues in the role of enzyme replacements. The book is of primary interest to paediatric neurologists, developmental biochemists, neurochemical patholog:sts and those concerned with chemical aspects of mental retardation. It gives a well-balanced sample of the problems relating to genetic defects in cleaving complex carbohydrate linkages in general and, of course, the sphingolipidoses in fine detail. E. J. Thompson
Human Memory - - The Processing of Information, by G . R. L o f t u s a n d E. F. L o f t u s , xii ÷
179 pages, 91 i l l u s t r a t i o n s , 6 table~, L a w r e n c e E r l b a u m A s s o c i a t e s , H i l l d a l e ,
N . J . , d i s t r i b u t e d by J o h n W i l e y a n d Sons, Ltd., C h i c h e s t e r , 1976, £ 6.60. U S $ 12.25. This book surveys attempts at the experimental unravelling of the nature of memory. An informationprocessing theoretical point of view provides a helpful framework within which to organize the data. The questions asked concern the storage, coding, transfer, loss and retrieval of information and key experiments are described in support of the explanations offered. In this way quite an advanced level of treatment is achieved without attempting a comprehensive review. Some active controversial issues are by-passed but would soon be encountered by anyone following up the well chosen references. Several chapters deal in succession with the evidence for and nature of the initial very brief sensory storage of unanalysed information, selective transfer to a limited capacity, less brief, but still short-term store of mainly acoustically coded information, and further transfer, involving organised forms of semantic coding, to a long-term store. Retrieval and loss from these stores are discussed as are the important differences between recall and recognition and between the remembering of events and of stored conceptual knowledge. A final chapter is devoted to practical applications of memory research in education, law, human engineering and clinical assessment. The clinical section is not very impressive and clinical evidence gains scant attention in the main chapters. The neurobiologist will not find in this book, nor in this field of study, a functional analysis of processes which can be closely correlated with his knowledge of brain structure and physiological functioning. He will find a well argued and readable account of phenomena which he will ultimately need to reconcile with his own studies. Psychologists will find an excellent introduction to the study of memory which is likely to become the prescribed text for many academic courses on this topic. H. Gwynne Jones