Invited Speakers
1-C
What we did and what we do
J. Egozcue
Professor Emeritus of Cell Biology, Universitat
Auto© nomade Barcelona, Spain
To commemorate the 50th anniversary of the ¢rst
accurate description of the human chromosome num-
ber, I will try to put into perspective the technologies
used in the earlier years, and the ones which are
available to us nowadays. First, I will pay a tribute to
some of our predecessors, such as T.S. Painter, who
established that our 2n was 46, to later change his mind
to 48, with some unexpected consequences, or Elisabeth
H. Slifer, who ¢rst tried the e¡ects of anisotonic solu-
tions to improve chromosome spreads. Then, we will
reach 1953 and the hypotonic miracle, which allowed
Tjio and Levan to con¢rm our 2n at 46, contempor-
aneously with Ford and Hamerton, who reached the
same conclusion using testicular material. Finally, we
will browse the developments which have taken place in
three cytogenetics ¢elds which I have chosen for reasons
of a¤nity: chromosome evolution, the cytogenetics of
reproduction and the cytogenetics of hematological
neoplasias. My lecture will try to demonstrate that, in
spite of the spectacular developments of molecular
biology, the chromosome, as the support of all activities
which take place in our genome, will always be the
essential element to map, de¢ne, correlate, interpret
and understand any genetic process.
2-C
Clinical ¢ndings in autosomal chromosome
aberrations
A. Schinzel
University of Zurich, Institute of Medical Genetics,
Switzerland
Autosomal chromosome aberrations are accompanied
by a characteristic phenotype which includes, as major
features, a pattern of minor dysmorphic signs, a pattern
of major organ malformations, mental de¢ciency and
impaired growth both pre- and postnatally. Many aber-
rations are associated with a speci¢c behavioural pro¢le.
Further characteristic ¢ndings include diminished and
delayed puberty and thus a long period of growth and
early senescence. If a deletion includes a gene with
haploinsu¤ciency, chromosome aberrations may also
show features of dominant or X-linked conditions.
Malformations frequently present in chromosome aber-
rations are those which are common in general and, if
present as isolated ¢ndings, usually follow multifactorial
inheritance.Dysmorphic¢ndingsmainlyconcernfacies,
genitalia and distal limbs and undergo marked changes
with age.
A review of further characteristic of autosomal chro-
mosome aberrations will be presented.
3-C
Autosomal imbalance and copy number
variation without phenotypic e¡ect
J. Barber
Wessex Regional Genetics Laboratory, UK
Directly transmitted cytogenetically visible anomalies
can be divided into unbalanced chromosome abnormal-
ities (UBCAs) and euchromatic variants (EVs).
UBCAs involve heterozygous single copy number
changes of segments larger than 2 Mb that do not vary
in the normal population. Among 130 UBCA families,
most of the UBCAs were unique to each family and 77/
130 (58%) were associated with a consistently mild
phenotype. In 30/130 (23%) families, the a¡ected pro-
band had the same UBCA as other normal family
members. In 23/130 (18%) families, ascertained mostly
at prenatal diagnosis, UBCAs with an average size of
almost 10 Mb had no detectable phenotypic e¡ect.
Regions of low gene density were involved in 3 of the 4
UBCAs reported in more than one normal family.
All the established cytogenetic EVs of 8p23.1, 9p12,
9q12, 15q11.2 and 16p11.2 have been described in
multiple independent families and most involve copy
number variation of paralogous gene and pseudogene
cassettes of less than 2Mb; these are polymorphic in the
normal population and only reach the cytogenetic level
when copy number is high. These EVs do not have
signi¢cant phenotypic consequences and the co-segrega-
tion of an EV with mild phenotypic anomalies in 2/70
(3%) families was thought to be coincidental. However,
EVs may yet be found to a¡ect traits which show
continuous variation.
The 200 UBCA and EV families form the `Chromo-
some Anomaly Collection' at http://www.ngrl.org.uk/
Wessex/register. Further close collaboration between
medical and laboratory sta¡ will be essential to distin-
guish clinicallysilent variation from pathogenic rearran-
gement.
ChromosomeResearch.2005; 13 (Supplement 1):1^16.
# 2005 Springer.PrintedintheNetherlands