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The clinical picture of diseases, caused by trinucleotide repeats,
such as fragile X syndrome, myotonic dystrophy, Huntington's
disease and forms of spinocerebella ataxia, provide the starting
point for this authoritative review volume.
The book proceeds to integrate the current understanding of the
molecular pathologies of these diseases, their genotype-phenotype
relationships, the mutational processes involved and the laboratory
and clinical issues relating to genetic testing for these
disorders.
Clinicians and researchers in genetics, neuroscience, pediatrics
and psychiatry will all benefit from the comprehensive overviews
contributed by recognized world experts.
It is a pleasure to send Dr. Hayden's monograph on its way to as
yet unknown but hopefully widespread destinations with all the
valedictions a Foreword may contain. Since I met the author in Cape
Town in 1978 I have been struck, on numerous occasions, by the
fortuitous combination of an inquisitive mind, a creative drive, a
sharp awareness of the historical and social setting of phenomena,
and a solid discipline which his personality displays behind a
good-natured laugh. If a tree is known by its fruits, both Dr
Hayden's PhD thesis and the present monograph afford quite an
insight into the auctor intellectualis. The amalgamation of the
terrible mise ry behind scientifIc facts and the elegantly artistic
presentation of this book will leave none of its readers
unperturbed. It reminds me of Nietzsche's 'Denn das Schone ist
nichts als des Schrecklichen Anfang, und wir bewundern es so weil
es gelassen verschmaht uns zu zerstoren' (Beauty is but Horror's
beginning, and we admire it because it resignedly spurns to destroy
us). The book is a denial, a testimony against Juvenal's spurious
'Sternmata quid faciunt ... ' (of what value are pedigrees). For it
is the very genetical prolongation of misery over the centuries
that brought Huntington's chorea to South Africa, Australia and the
USA from the shores of sea-faring seventeenth-century England and
Holland."
My personal witness, as the multiple keyboard player/back-ground
singer and one of the Creatures of the Street, in the band
JOBRIATH, during our ten month journey, starting with my audition,
then the band's rehearsal for the Midnight Special and subsequent
national tour, all against the homophobic backlash which Bruce
Wayne Campbell, a.k.a. Jobriath's own self-outing made him a "dead
man walking." We played on. Even the gay community resented him
being paraded as their "'poster child, ' demonstrating attributes
they loathed in themselves." Though the rest of the band was
straight, so extreme was this hostility, we were specifically not
booked in Cleveland, Cincinnati and Detroit, for fear of us being
physically harmed. Even in New York, we were booed off the stage at
the Nassau Coliseum for being "fagots." Jerry Brandt, was
Jobriath's larger-than-life manager. Like Colonel Parker to Elvis,
Jerry created a huge hype machine that took America and Britain by
storm: "Elvis, The Beatles, Jobriath " With only fifty thousands
dollars and a single album record deal, Jerry created a
half-a-million dollar media blitz and a mountain of jealousy that
would subsequently be impossible to overcome.
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