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Nerves and mechanoreceptors
RE-VIEW
B. Brooks and K. Fischbeck - Spinaland bulbarmuscularatrophy p a t h o l o g i c features (Table 1). T h e y are all progressive, a d u l t - o n s e t , h e r e d i t a r y n e u r o d e g e n e r a t i v e diseases. All h a v e s h o w n e v i d e n c e of g e n e t i c instability. F u r t h e r m o r e , in e a c h case, t h e age at o n s e t of s y m p t o m s correlates w i t h t h e l e n g t h of t h e C A G repeat. In all b u t M a c h a d o - J o s e p h disease, t h e g l u t a m i n e repeat is located n e a r t h e a m i n o t e r m i n u s of t h e p r o t e i n . T h e g e n e s t h a t are m u t a t e d in t h e s e o t h e r diseases are n o v e l , a n d t h e i r n o r m a l f u n c t i o n s are u n k n o w n . At least in t h e case of t h e g e n e for H u n t i n g t o n ' s disease, t h e express i o n p a t t e r n is w i d e s p r e a d , a n d c a n n o t a l o n e a c c o u n t for t h e selective n e u r o n a l d e g e n e r a t i o n zs. The c o m m o n features of this e m e r g i n g f a m i l y of i n h e r i t e d n e u r o d e g e n e r a t i v e diseases t h a t are c a u s e d b y e x p a n d e d C A G repeats suggest a c o m m o n m e c h a n i s m of p a t h o genesis. T h e h y p o t h e s e s t h a t h a v e b e e n p r o p o s e d for t h e p a t h o p h y s i o l o g y of SBMA are also a p p l i c a b l e to t h e s e o t h e r diseases. U n d o u b t e d l y , a d v a n c e s m a d e i n s t u d y i n g e a c h of t h e s e diseases will h e l p to e l u c i d a t e t h e m e c h a n i s m in t h e o t h e r diseases. Selected references 1 Kennedy, W.R., Alter, M. and Sung, J.H. (1968) Neurology 18, 671-680
2 Trifiro, M.A., Kazemi-Esfarjani, P. and Pinksy, L. (1994) Trends EndocrinoL Metab. 5, 416-421 3 Sobue, G. et al. (1989) Brain 112, 209-232 4 Nagashima, T. et al. (1988) J. NeuroI. Sci. 87, 141-152 5 La Spada, A.R. et al. (1991) Nature 352, 77-79 6 Tsai, M-J. and O'Malley, B.W. (1994) Annu. Rev. Biochem. 63, 451~t86 7 La Spada, A.R. et al. (1992) Nat. Genet. 2, 301-304 8 Clancy, A.N., Bonsall, R.W. and Michael, R.P. (1992) Life Sci. 50, 409-417 9 Matsuura, T. et al. (1993) NeuroscL Lett. 158, 5-8 10 Simental, J.A. et al. (1991) J. Biol. Chem. 266, 510-518 11 Kazemi-Esfarjani, P. et al. (1994) Am. J. Hum. Genet. 55 (Suppl.), A18 12 Mhatre, A.N. et al. (1993) Nat. Genet. 5, 184-188 13 Pinksy, L. et al. (1992) Ctin. Invest. Med. 15, 456-472 14 Yu, W.A. (1989) J. NeuroscL 9, 3908-3914 15 Shughrue, P.J. and Dorsa, D.M. (1994) Endocrinology 143, 1321-1328 16 Perutz, M.F. et al. (1994) Proc. Natl Acad. Sci. USA 91, 5355-5358 17 Mitchell, P.J. and Tijan, R. (1989) Science 245, 371-378 18 Green, H. (1993) Cell 74, 955-956 19 Bingham, P.M. et al. (1995) Nat. Genet. 9, 191-196 20 The Huntington's Disease Collaborative Research Group (1993) Cell 72, 971-983 21 Banff, S. et al. (1994) Nat. Genet. 7, 513-520 22 Koide, R. et al. (1994) Nat. Genet. 6, 9-13 23 Nagafuchi, S. et al. (1994) Nat. Genet. 6, 14-18 24 Kawaguchi, Y. et al. (1994) Nat. Genet. 8, 221-227 25 Hoogeveen, A.T. et al. (1993) Hum. MoL Genet. 2, 2069-2073
Acknowledgements Work in the authors' laboratory was funded in part by the Muscular Dystrophy Association, the March of Dimes, the ALS Association, and the National Institutes of Health. The authors thank Diane E. Merry and Henry Paulson for their thoughtful reading of the manuscript, and for many helpful discussions.
BOOK REVIEW Nerves and Mechanoreceptors by Jirina Zelena, Chapman and Hall, 1994. £45.00 (355 pages) ISBN 0 412 43430 X This is a monograph written by a wellknown, established worker in the field of neural development and regeneration. It therefore represents an authoritative account of what, in the past, has been a field littered with confusing and contradictory observations. So, I was looking forward eagerly to receiving a balanced and summarizing view. I was a little disappointed in my expectations. The descriptions of many of the areas of controversy do not always leave the reader with a clear view of the current state of the field. Here, it is necessary to bear in mind that it is a particularly difficult field to summarize, one that is undergoing rapid expansion and change. The advent and development of tissue culture techniques, and the application of histochemical and immunocytochemical methods to the analysis of the various factors that are involved in the growth and development of neurones has opened up what probably represents a whole new field. The days of such simple, old-fashioned experiments as cutting or crushing a nerve, and then observing what happens, are probably gone. Now the approach is more likely to use immunological or molecular biological techniques. In that sense, the book provides a useful historical perspective of the subject, while bringing the reader up to date in many areas. The general emphasis in. Nerves and Mechanoreceptors is on structure rather than
function, presumably reflecting the interests of the author. There is an opening chapter giving the reader an introduction to primary sensory neurones and the factors that are involved in their growth and regeneration. This is followed by six chapters, each dedicated to one particular type of receptor. The structure of these chapters is unusual. Each opens with an account of development of the particular receptor, and the effects of various kinds of experimental manipulations on developmental processes. Then comes a section, in small print, ~that describes the structure and physiology of the adult receptor. The results of experimental manipulations of adult receptors are discussed next, and the chapter finishes with a summarizing section. These summaries, incidentally, are very useful to the uninformed reader who is looking for a brief synopsis of the chapter contents. My preference would have been to have discussed the adult receptor first, and then go into the details of development and regeneration. I understand, of course, that the author is committed to achieving a delicate balance, not letting details of the adult condition dominate the chapter, yet providing sufficient information about the adult to make the description of the various experiments that relate to development and regeneration comprehensible to someone who is unfamiliar with the field.
Turning to some points of detail, one of the interesting consequences of peripheralnerve section is that several days later, the growing tip of the nerve proximal to the section becomes sensitive to mechanical stimuli, and this mechanosensitivity is different for skin and muscle nerves. It is a pity that this finding has been omitted from the book as it has important consequences for the interpretation of experiments on reinnervation and cross-reinnervation. In the account of regenerating nerve, it was not made clear that the process of reinnervation of skin and muscle is an essentially random affair. I was pleased to see that the deficiencies of the model for sensory transduction in tendon organs, using lateral compression of nerve endings, were pointed out. For tendon organs, as for muscle spindles, the evidence supports simple stretch of the nerve endings as the adequate stimulus for the generation of activity. To conclude, this is a detailed, comprehensively referenced, monograph with many useful diagrams and photographs that will be of great help to research students and teachers who want to be brought up to date in this rather specialized field. The few shortcomings are outshone by the rigorous approach and demonstrated scholarship of the work.
U w e Proske Dept of Physiology, Monash University, Clayton, Victoria 3168, Australia.
TINS Vol. 18, No. 10, 1995
461
B. Brooks and K. Fischbeck - Spinaland bulbarmuscularatrophy p a t h o l o g i c features (Table 1). T h e y are all progressive, a d u l t - o n s e t , h e r e d i t a r y n e u r o d e g e n e r a t i v e diseases. All h a v e s h o w n e v i d e n c e of g e n e t i c instability. F u r t h e r m o r e , in e a c h case, t h e age at o n s e t of s y m p t o m s correlates w i t h t h e l e n g t h of t h e C A G repeat. In all b u t M a c h a d o - J o s e p h disease, t h e g l u t a m i n e repeat is located n e a r t h e a m i n o t e r m i n u s of t h e p r o t e i n . T h e g e n e s t h a t are m u t a t e d in t h e s e o t h e r diseases are n o v e l , a n d t h e i r n o r m a l f u n c t i o n s are u n k n o w n . At least in t h e case of t h e g e n e for H u n t i n g t o n ' s disease, t h e express i o n p a t t e r n is w i d e s p r e a d , a n d c a n n o t a l o n e a c c o u n t for t h e selective n e u r o n a l d e g e n e r a t i o n zs. The c o m m o n features of this e m e r g i n g f a m i l y of i n h e r i t e d n e u r o d e g e n e r a t i v e diseases t h a t are c a u s e d b y e x p a n d e d C A G repeats suggest a c o m m o n m e c h a n i s m of p a t h o genesis. T h e h y p o t h e s e s t h a t h a v e b e e n p r o p o s e d for t h e p a t h o p h y s i o l o g y of SBMA are also a p p l i c a b l e to t h e s e o t h e r diseases. U n d o u b t e d l y , a d v a n c e s m a d e i n s t u d y i n g e a c h of t h e s e diseases will h e l p to e l u c i d a t e t h e m e c h a n i s m in t h e o t h e r diseases. Selected references 1 Kennedy, W.R., Alter, M. and Sung, J.H. (1968) Neurology 18, 671-680
2 Trifiro, M.A., Kazemi-Esfarjani, P. and Pinksy, L. (1994) Trends EndocrinoL Metab. 5, 416-421 3 Sobue, G. et al. (1989) Brain 112, 209-232 4 Nagashima, T. et al. (1988) J. NeuroI. Sci. 87, 141-152 5 La Spada, A.R. et al. (1991) Nature 352, 77-79 6 Tsai, M-J. and O'Malley, B.W. (1994) Annu. Rev. Biochem. 63, 451~t86 7 La Spada, A.R. et al. (1992) Nat. Genet. 2, 301-304 8 Clancy, A.N., Bonsall, R.W. and Michael, R.P. (1992) Life Sci. 50, 409-417 9 Matsuura, T. et al. (1993) NeuroscL Lett. 158, 5-8 10 Simental, J.A. et al. (1991) J. Biol. Chem. 266, 510-518 11 Kazemi-Esfarjani, P. et al. (1994) Am. J. Hum. Genet. 55 (Suppl.), A18 12 Mhatre, A.N. et al. (1993) Nat. Genet. 5, 184-188 13 Pinksy, L. et al. (1992) Ctin. Invest. Med. 15, 456-472 14 Yu, W.A. (1989) J. NeuroscL 9, 3908-3914 15 Shughrue, P.J. and Dorsa, D.M. (1994) Endocrinology 143, 1321-1328 16 Perutz, M.F. et al. (1994) Proc. Natl Acad. Sci. USA 91, 5355-5358 17 Mitchell, P.J. and Tijan, R. (1989) Science 245, 371-378 18 Green, H. (1993) Cell 74, 955-956 19 Bingham, P.M. et al. (1995) Nat. Genet. 9, 191-196 20 The Huntington's Disease Collaborative Research Group (1993) Cell 72, 971-983 21 Banff, S. et al. (1994) Nat. Genet. 7, 513-520 22 Koide, R. et al. (1994) Nat. Genet. 6, 9-13 23 Nagafuchi, S. et al. (1994) Nat. Genet. 6, 14-18 24 Kawaguchi, Y. et al. (1994) Nat. Genet. 8, 221-227 25 Hoogeveen, A.T. et al. (1993) Hum. MoL Genet. 2, 2069-2073
Acknowledgements Work in the authors' laboratory was funded in part by the Muscular Dystrophy Association, the March of Dimes, the ALS Association, and the National Institutes of Health. The authors thank Diane E. Merry and Henry Paulson for their thoughtful reading of the manuscript, and for many helpful discussions.
BOOK REVIEW Nerves and Mechanoreceptors by Jirina Zelena, Chapman and Hall, 1994. £45.00 (355 pages) ISBN 0 412 43430 X This is a monograph written by a wellknown, established worker in the field of neural development and regeneration. It therefore represents an authoritative account of what, in the past, has been a field littered with confusing and contradictory observations. So, I was looking forward eagerly to receiving a balanced and summarizing view. I was a little disappointed in my expectations. The descriptions of many of the areas of controversy do not always leave the reader with a clear view of the current state of the field. Here, it is necessary to bear in mind that it is a particularly difficult field to summarize, one that is undergoing rapid expansion and change. The advent and development of tissue culture techniques, and the application of histochemical and immunocytochemical methods to the analysis of the various factors that are involved in the growth and development of neurones has opened up what probably represents a whole new field. The days of such simple, old-fashioned experiments as cutting or crushing a nerve, and then observing what happens, are probably gone. Now the approach is more likely to use immunological or molecular biological techniques. In that sense, the book provides a useful historical perspective of the subject, while bringing the reader up to date in many areas. The general emphasis in. Nerves and Mechanoreceptors is on structure rather than
function, presumably reflecting the interests of the author. There is an opening chapter giving the reader an introduction to primary sensory neurones and the factors that are involved in their growth and regeneration. This is followed by six chapters, each dedicated to one particular type of receptor. The structure of these chapters is unusual. Each opens with an account of development of the particular receptor, and the effects of various kinds of experimental manipulations on developmental processes. Then comes a section, in small print, ~that describes the structure and physiology of the adult receptor. The results of experimental manipulations of adult receptors are discussed next, and the chapter finishes with a summarizing section. These summaries, incidentally, are very useful to the uninformed reader who is looking for a brief synopsis of the chapter contents. My preference would have been to have discussed the adult receptor first, and then go into the details of development and regeneration. I understand, of course, that the author is committed to achieving a delicate balance, not letting details of the adult condition dominate the chapter, yet providing sufficient information about the adult to make the description of the various experiments that relate to development and regeneration comprehensible to someone who is unfamiliar with the field.
Turning to some points of detail, one of the interesting consequences of peripheralnerve section is that several days later, the growing tip of the nerve proximal to the section becomes sensitive to mechanical stimuli, and this mechanosensitivity is different for skin and muscle nerves. It is a pity that this finding has been omitted from the book as it has important consequences for the interpretation of experiments on reinnervation and cross-reinnervation. In the account of regenerating nerve, it was not made clear that the process of reinnervation of skin and muscle is an essentially random affair. I was pleased to see that the deficiencies of the model for sensory transduction in tendon organs, using lateral compression of nerve endings, were pointed out. For tendon organs, as for muscle spindles, the evidence supports simple stretch of the nerve endings as the adequate stimulus for the generation of activity. To conclude, this is a detailed, comprehensively referenced, monograph with many useful diagrams and photographs that will be of great help to research students and teachers who want to be brought up to date in this rather specialized field. The few shortcomings are outshone by the rigorous approach and demonstrated scholarship of the work.
U w e Proske Dept of Physiology, Monash University, Clayton, Victoria 3168, Australia.
TINS Vol. 18, No. 10, 1995
461