- Email: [email protected]
Dystrophin: Gene, protein and cell biology
as well as the role of methylation patterns, telomeric maintenance, DNAdamage signalling through the DNA-dependent protein kinase, and the role of aberrant V@)J DNArecombination in cancers of the haema:opcietic system. Of course, such a title would be impossible without including chapters on the gene characteristics and multifunctional properties of the tumour suppressor ~53. Furthermore, ceil-cycle control mechanisms, genetic disorders predisposing for cancer, and chromosomal rearrangements associated with cancer have also been covered. It seems that we are now at a stage approaching a molecular understanding of some of the individual processes involved in cancer development, however, still !acking important pieces required to put things together in a more complete, overall picture. All chapters are written by experts in their respective fields and are limited in length and uniform in style to make a very consistent volume, in spite of the many different authors involved.
It is a common problem these days to keep up to date on recent developments in molecular and cell biology as a whole. With the new information retrieval services available, it is quite easy to find relevant literature, but difficult to distinguish between ‘good and bad reading. Meeting proceedings and reports are usually without much value, as people do not present interesting work in books any more, because of the limited credit obtained. The alternative to many excellent reviews spread around in many different regular journals are books like this one, comprising a wider range of reviews expressing the personal views from leading scientists in the various areas. For me personally, chapters from Larry Loeb on ‘Many notations in Ca and from Bryn Bridges on the adaptive mutation phenomenon were particularly inspiring, not only because of my interest in the subjects they address, but also from their unique and personal ways of approaching the subjects. Others may find different chapters more enlightening, of course, depending on the scientific
among scientists, which often precludes
meaningful exchanges! The done well in ~ttting togeth remarkably broad-based yet cohesive book, and I am sure it will prove useful to newcomers in the field. The book Is organized in I1 chapters,
Dystrophin: Gene, Protein and Cell Biology
and ’ ringing’ of the book represents an ever, ource of inf~rrna~~o~for scientists wanting to get acquainted with the different rapidly moving areas in the field of mQlec~fa~ cancer research, and to find key references for further reading.
from better indexing. However, the overall pression is excellent aradmuch cre itor, in ~ar~ic~~~~,who is mark on the volume.
Medical Microbiology, University of Osio, The National Hospital, N-0027 Oslo, Norway.
ons of many e.g. the French arc~~~yca~ topics were not adequ the book, either. It is ~a~tc~~~rfy ironic to
restrict c~~t~ib~ta~s to j&t these two natiions because if it wasn’t for the efforts
its in
5 This book covers the emerging field of dystrophfn biology and many of the advances that have been made over the past decade, in ol;r understanding of the etiology and pathogenesis of Duchenne muscular dystrophy @MD). In addition, the editors have wisely included chapters on some basic issues involving muscle biology (e.g. regeneration) that are integral to understanding the function of dystrophtn and the patho-physiology that ensues dystrophin deficiency. Based on the embarrassment of riches in terms of the quality of researchers that have contributed to this book, it is not surprising that individual chapters in this book are well-written, well-researched, insightful and current. Also, some contributors have chosen to address issues crftical to understanding the basic biology of DMDin this book, which are unfortunately often left unaddressed in other forms of communication, e.g. in journals, perhaps owing to pressure of space: and at meetings, perhaps owing to the existence of dystrophin-size egos
protein and so on: chapte surely help bring the newcomer or ~~t~reste~ onlooker up to speed, in terms of established facts concerning dystrophin bioiogy, and may even be of use to jaded Pfs wishing to brush up on their facts and references. The last six chapters, I felt were slightly different in character, and explored issues, enigmas and problems in the field and provided hints to their solutions: I particularly enjoyed the insightful chapters on the path+physiology, regeneration and animal models of dystrophin deficiency. 1am sure that this section of the book will provide food for thought for many researchers, and may help clarify
useful to newcomers to the field rather than established investigators, what are some of the shortcomings of this book? First, I was somewhat surprised that the only contributors listed were based in the UK or in the USA.Whether, this occurred by chance or design I cannot comment, however, it is surprising considering the global nature of research in this field, as exemplified by the seminal
Second, I was sur~rfsed at the omisslan of charters on the natural history of the disease and the bisto-pathological consequences of DMDin humans. These chapters are particularly important, as dystrophin deficiency has been studted rather extensively in humans (for over a century!), but not quite so carefully in animal models. Indeed, there exists a continuing set of problems in analysing animal models. As Professor Partridge rightly, points out on p. 312, ‘The history of the mdw mouse is an illuminating record of the inadequacies of our ability to survey, describe, and characterize a disease model...(del)...Since the mdx mouse model itself provides no reasonable excuse, one must look to the pressures that encourage rapid scientific publication and postpone the conduct of careful, systematic chronic studies to a position of last resort’. As a book is somewhat protected from pressures of space and rapid communication, I was surprised at I he lack of information on the natural histary of the disease; something that goes hand-in-hand with studying dystrophin. I searched
BOOKREVIEWS
T~8S 22 = DECEMBER 1997 [ndividualchapters, but could not find even basic discussions on ~nndamenta] features associated with dystrophy such as EKG findings, a description of Cower's sign or even discussions on clinical variability. Knowing about these features would benefit a newcomer enormously and a~d them when tr3~ngto evaluate the significance of newer therapeutic strategies, e.g. clinical variability in Becket's, while evaluating global applicability of the mini-gone approach. To paraphrase what Sir Wfl~i~mOsier said about patients and books: if studying DMD without b o o ~ is sailingthe uncharted seas, studying dystrophin without studying DMD is not to go to sea at all! Third, I wou~dhave really likedto see some chapters dealing with the history of research in this field (perhaps an introductory chapter, researched and written by the authors), highlighting
important Mndmarksand commentingon their significanceoThis wo~|d real~yhelp cement the book together, acknowledge key contributions and references that may have been omitted by individual authors, as well as impart to the reader the sense that the discovery of dystrophin was, and dystrophin biology continues to be, a fie~d of exciting, groundbreaking discoveries. As it stands, even alter reading the book from cover to cover, a reader would not be able to appreciate the contribution of Duchenne in describing the natural history and pathology of the disease, and indeed his contribution in mapping it to the X chromosome, why BB's DNA was important to the PERT strategy used to identify DMD deletions and clone the DMD gene, or even find references to the above mentioned pivotal studies anywhere in the book[ Fourth, ~believe that this book could improve with careful proofing (e.g. on
p. 190, it should surely be 37°C rather ~han 370°C), publishing a list ot web~ink~ where one can find more detailed informationon various topics (e.~. OM~M or MDAgroups across the globe),adding adc'.itionalchapters mentionedabove, and publishing the contributers fax/ phone numbers and Email addresses, rather than 'snail mall' addresses. [~ conclusion, Brown and Lucy deserve credit for generating this enjoyable book and I look forward to seeing future (and hopefully more comprehensive) edition(s) of this book.
laking a good job of the [mpossibme
basis of disease, on which protein targeting now impinges. The interests of both groups c~, not necessarily coincide. The format Js familiar' six chapters by different a~ti'hors, each about 20 pages, which sum to an area not so much greater than Alberts d e v o t ~ to the same topics. This format, then, imposes the first constraints, and out go chloroplasts and everything to do with prokaryotes (to understandable howls of complaint from some quarters). 'Fairness' to the authors also leads to some apparent anomalies: 20 pages on peroxisome targeting seems generous, but the same to cover targeting and traffic between and through the endoplasmic reticulum and Golgi necessitates heroic [eats oi literary anorexia from the author. The multiauthor format imposes a further constraint: having persuaded the authors, each of whom is amply qualified in their topic, to produce a chapter, it is hardly possible to rewrite it to enforce a uniformity of style and approach. Thus, chapters vary considerably in these respects. The first desirable feature is a clear account of the problems, followed by an honest statement of the current position. One does not wish to be bogged down in the uninterpreted detail of the latest papers, and worst of all this is hardly the place to release the authors' unpublished data, but sadly there are offenders on both counts. This reviewer's prize for narrative clarity and intellectual coherence. however, goes to Romisch and Corsi. Perhaps their topic, translocation into the endoplasmic reticulum, has progressed more rationally than others, through cell biology and biochemistry to biophysics: but it has also been blessed with its fair share of rampaging controversy, so credit must go to the authors.
By contrast, 'rational progress' is not necessarily the first term that comes to mind [n describing the recent history of vesicle traffic, and understandably the three chapters in this area take rather different tacks. Ktistakis and Roth approach their subject, protein sorting during endocytosis, with commendable boldness arm conclude with an invigorating piece of iconoclasm, although perhaps the chapter could have been stamped 'Adults only' to protect the aforementioned undergraduates. But everyone (the authors, the editor, presumably the publishers) knows the frightening problem with a book of this sort. |~ it really possi tie to be at the irontiers of molecular biology and describe targeting to the nucleus without using the terms importin or karyopherin, or traffic from the endoplasmic reticulum to the Golgi without the COPII coat? Check the dates. For bound-paper publishing from commissioning an article to publishing a TiBS review can take two years. In that time, active research areas can undergo not just substantial development, but, as in the case of vesicle trafficking, near-Maoist upheaval. The authors' only crime is to lack a supernatural degree of prescience that would put all of us out of business. in sum, then, this volume is a collection of summaries, in varying styles but all abundantly referenced, of a dynamic and expanding field. To those outside the field it offers some useful starting points to becoming acquainted. To the smaller audience within it might serve a rather different purpose: as a salutary reminder of how much can change in a very short time.
Protein Targeting: Frontiers in Molecular Biolo[~ edited by Stella M, H~ffey, IRL Press, 1996. £29.95 (xi# + 234 pages) ISBN 0 19 963561 7 It's those hard little eyes that get you. "Protein targeting and membrane traffic is an important and mteresting topic. The best textbook is AIberts/Damell/whatever, but unfortunately it's a bit out of date, and some of us think that parts of it are, urn, wrong.' And the angry stares tell you that the less-able students in the class have decided that at least one of their sources of information is telling lies. (No problem with the brighter ones, of course, who realised this some time ago.) For those of us who have been in this position, and wished for something in between the cartoon idyll of modern cell biology textbooks and the writhing mess of experimental reality, does Protein Targeting answer our prayers? The first thing to say, in fairness to all concerned, is that no-one claims that either the book or the Frontiers in Molecular Biology series is particularly aimed at advanced undergraduates. Who, then, might constitute the potential readership? The first group are those who work in the field and are not too busy arguing among themselves to read books; the second and much larger audience are those whose interests are in the growing list of topics, from signal transduction and gone expression to the molecular
T~VWR $. KHURANA Department of Clinical Biochemistry, Glostrup Hospital, University Copenhagen Medical School, DK 2600. Glostrup, Denmark. Email: [email protected]
JOHN ARMSTRONG School of Biological Sciences, University of Sussex, Falmer, Brighton, UK BN19QG.
497
It is a common problem these days to keep up to date on recent developments in molecular and cell biology as a whole. With the new information retrieval services available, it is quite easy to find relevant literature, but difficult to distinguish between ‘good and bad reading. Meeting proceedings and reports are usually without much value, as people do not present interesting work in books any more, because of the limited credit obtained. The alternative to many excellent reviews spread around in many different regular journals are books like this one, comprising a wider range of reviews expressing the personal views from leading scientists in the various areas. For me personally, chapters from Larry Loeb on ‘Many notations in Ca and from Bryn Bridges on the adaptive mutation phenomenon were particularly inspiring, not only because of my interest in the subjects they address, but also from their unique and personal ways of approaching the subjects. Others may find different chapters more enlightening, of course, depending on the scientific
among scientists, which often precludes
meaningful exchanges! The done well in ~ttting togeth remarkably broad-based yet cohesive book, and I am sure it will prove useful to newcomers in the field. The book Is organized in I1 chapters,
Dystrophin: Gene, Protein and Cell Biology
and ’ ringing’ of the book represents an ever, ource of inf~rrna~~o~for scientists wanting to get acquainted with the different rapidly moving areas in the field of mQlec~fa~ cancer research, and to find key references for further reading.
from better indexing. However, the overall pression is excellent aradmuch cre itor, in ~ar~ic~~~~,who is mark on the volume.
Medical Microbiology, University of Osio, The National Hospital, N-0027 Oslo, Norway.
ons of many e.g. the French arc~~~yca~ topics were not adequ the book, either. It is ~a~tc~~~rfy ironic to
restrict c~~t~ib~ta~s to j&t these two natiions because if it wasn’t for the efforts
its in
5 This book covers the emerging field of dystrophfn biology and many of the advances that have been made over the past decade, in ol;r understanding of the etiology and pathogenesis of Duchenne muscular dystrophy @MD). In addition, the editors have wisely included chapters on some basic issues involving muscle biology (e.g. regeneration) that are integral to understanding the function of dystrophtn and the patho-physiology that ensues dystrophin deficiency. Based on the embarrassment of riches in terms of the quality of researchers that have contributed to this book, it is not surprising that individual chapters in this book are well-written, well-researched, insightful and current. Also, some contributors have chosen to address issues crftical to understanding the basic biology of DMDin this book, which are unfortunately often left unaddressed in other forms of communication, e.g. in journals, perhaps owing to pressure of space: and at meetings, perhaps owing to the existence of dystrophin-size egos
protein and so on: chapte surely help bring the newcomer or ~~t~reste~ onlooker up to speed, in terms of established facts concerning dystrophin bioiogy, and may even be of use to jaded Pfs wishing to brush up on their facts and references. The last six chapters, I felt were slightly different in character, and explored issues, enigmas and problems in the field and provided hints to their solutions: I particularly enjoyed the insightful chapters on the path+physiology, regeneration and animal models of dystrophin deficiency. 1am sure that this section of the book will provide food for thought for many researchers, and may help clarify
useful to newcomers to the field rather than established investigators, what are some of the shortcomings of this book? First, I was somewhat surprised that the only contributors listed were based in the UK or in the USA.Whether, this occurred by chance or design I cannot comment, however, it is surprising considering the global nature of research in this field, as exemplified by the seminal
Second, I was sur~rfsed at the omisslan of charters on the natural history of the disease and the bisto-pathological consequences of DMDin humans. These chapters are particularly important, as dystrophin deficiency has been studted rather extensively in humans (for over a century!), but not quite so carefully in animal models. Indeed, there exists a continuing set of problems in analysing animal models. As Professor Partridge rightly, points out on p. 312, ‘The history of the mdw mouse is an illuminating record of the inadequacies of our ability to survey, describe, and characterize a disease model...(del)...Since the mdx mouse model itself provides no reasonable excuse, one must look to the pressures that encourage rapid scientific publication and postpone the conduct of careful, systematic chronic studies to a position of last resort’. As a book is somewhat protected from pressures of space and rapid communication, I was surprised at I he lack of information on the natural histary of the disease; something that goes hand-in-hand with studying dystrophin. I searched
BOOKREVIEWS
T~8S 22 = DECEMBER 1997 [ndividualchapters, but could not find even basic discussions on ~nndamenta] features associated with dystrophy such as EKG findings, a description of Cower's sign or even discussions on clinical variability. Knowing about these features would benefit a newcomer enormously and a~d them when tr3~ngto evaluate the significance of newer therapeutic strategies, e.g. clinical variability in Becket's, while evaluating global applicability of the mini-gone approach. To paraphrase what Sir Wfl~i~mOsier said about patients and books: if studying DMD without b o o ~ is sailingthe uncharted seas, studying dystrophin without studying DMD is not to go to sea at all! Third, I wou~dhave really likedto see some chapters dealing with the history of research in this field (perhaps an introductory chapter, researched and written by the authors), highlighting
important Mndmarksand commentingon their significanceoThis wo~|d real~yhelp cement the book together, acknowledge key contributions and references that may have been omitted by individual authors, as well as impart to the reader the sense that the discovery of dystrophin was, and dystrophin biology continues to be, a fie~d of exciting, groundbreaking discoveries. As it stands, even alter reading the book from cover to cover, a reader would not be able to appreciate the contribution of Duchenne in describing the natural history and pathology of the disease, and indeed his contribution in mapping it to the X chromosome, why BB's DNA was important to the PERT strategy used to identify DMD deletions and clone the DMD gene, or even find references to the above mentioned pivotal studies anywhere in the book[ Fourth, ~believe that this book could improve with careful proofing (e.g. on
p. 190, it should surely be 37°C rather ~han 370°C), publishing a list ot web~ink~ where one can find more detailed informationon various topics (e.~. OM~M or MDAgroups across the globe),adding adc'.itionalchapters mentionedabove, and publishing the contributers fax/ phone numbers and Email addresses, rather than 'snail mall' addresses. [~ conclusion, Brown and Lucy deserve credit for generating this enjoyable book and I look forward to seeing future (and hopefully more comprehensive) edition(s) of this book.
laking a good job of the [mpossibme
basis of disease, on which protein targeting now impinges. The interests of both groups c~, not necessarily coincide. The format Js familiar' six chapters by different a~ti'hors, each about 20 pages, which sum to an area not so much greater than Alberts d e v o t ~ to the same topics. This format, then, imposes the first constraints, and out go chloroplasts and everything to do with prokaryotes (to understandable howls of complaint from some quarters). 'Fairness' to the authors also leads to some apparent anomalies: 20 pages on peroxisome targeting seems generous, but the same to cover targeting and traffic between and through the endoplasmic reticulum and Golgi necessitates heroic [eats oi literary anorexia from the author. The multiauthor format imposes a further constraint: having persuaded the authors, each of whom is amply qualified in their topic, to produce a chapter, it is hardly possible to rewrite it to enforce a uniformity of style and approach. Thus, chapters vary considerably in these respects. The first desirable feature is a clear account of the problems, followed by an honest statement of the current position. One does not wish to be bogged down in the uninterpreted detail of the latest papers, and worst of all this is hardly the place to release the authors' unpublished data, but sadly there are offenders on both counts. This reviewer's prize for narrative clarity and intellectual coherence. however, goes to Romisch and Corsi. Perhaps their topic, translocation into the endoplasmic reticulum, has progressed more rationally than others, through cell biology and biochemistry to biophysics: but it has also been blessed with its fair share of rampaging controversy, so credit must go to the authors.
By contrast, 'rational progress' is not necessarily the first term that comes to mind [n describing the recent history of vesicle traffic, and understandably the three chapters in this area take rather different tacks. Ktistakis and Roth approach their subject, protein sorting during endocytosis, with commendable boldness arm conclude with an invigorating piece of iconoclasm, although perhaps the chapter could have been stamped 'Adults only' to protect the aforementioned undergraduates. But everyone (the authors, the editor, presumably the publishers) knows the frightening problem with a book of this sort. |~ it really possi tie to be at the irontiers of molecular biology and describe targeting to the nucleus without using the terms importin or karyopherin, or traffic from the endoplasmic reticulum to the Golgi without the COPII coat? Check the dates. For bound-paper publishing from commissioning an article to publishing a TiBS review can take two years. In that time, active research areas can undergo not just substantial development, but, as in the case of vesicle trafficking, near-Maoist upheaval. The authors' only crime is to lack a supernatural degree of prescience that would put all of us out of business. in sum, then, this volume is a collection of summaries, in varying styles but all abundantly referenced, of a dynamic and expanding field. To those outside the field it offers some useful starting points to becoming acquainted. To the smaller audience within it might serve a rather different purpose: as a salutary reminder of how much can change in a very short time.
Protein Targeting: Frontiers in Molecular Biolo[~ edited by Stella M, H~ffey, IRL Press, 1996. £29.95 (xi# + 234 pages) ISBN 0 19 963561 7 It's those hard little eyes that get you. "Protein targeting and membrane traffic is an important and mteresting topic. The best textbook is AIberts/Damell/whatever, but unfortunately it's a bit out of date, and some of us think that parts of it are, urn, wrong.' And the angry stares tell you that the less-able students in the class have decided that at least one of their sources of information is telling lies. (No problem with the brighter ones, of course, who realised this some time ago.) For those of us who have been in this position, and wished for something in between the cartoon idyll of modern cell biology textbooks and the writhing mess of experimental reality, does Protein Targeting answer our prayers? The first thing to say, in fairness to all concerned, is that no-one claims that either the book or the Frontiers in Molecular Biology series is particularly aimed at advanced undergraduates. Who, then, might constitute the potential readership? The first group are those who work in the field and are not too busy arguing among themselves to read books; the second and much larger audience are those whose interests are in the growing list of topics, from signal transduction and gone expression to the molecular
T~VWR $. KHURANA Department of Clinical Biochemistry, Glostrup Hospital, University Copenhagen Medical School, DK 2600. Glostrup, Denmark. Email: [email protected]
JOHN ARMSTRONG School of Biological Sciences, University of Sussex, Falmer, Brighton, UK BN19QG.
497