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------=_NextPart_01C5C1F7.92B1B5D0 Content-Location: file:///C:/751B2C69/nickdennis240905(b)_files/pres.xml Content-Transfer-Encoding: quoted-printable Content-Type: text/xml; charset="utf-8" ------=_NextPart_01C5C1F7.92B1B5D0 Content-Location: file:///C:/751B2C69/nickdennis240905(b)_files/slide0001.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Klinefelter syndrome: a clinical geneticist’s view
Klinefelter syndrome: a clinical geneticist’s view
Nick Dennis
Wessex Clinical Genetics Service
Princess Anne Hospital
Southampton
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Harry Klinefelter described KS = in 1942
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Fyfaf7yf99UUUUWHc//Z ------=_NextPart_01C5C1F7.92B1B5D0 Content-Location: file:///C:/751B2C69/nickdennis240905(b)_files/slide0003.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Klinefelter syndrome: a clinical geneticist’s view
The presence= of an extra X chromosome i= n KS was first reported by = Patricia Jacobs
in 1959 =
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Chromosomes from a 46,XY male
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&s= ect;23 chromosomes from mother
&= sect;22 + X
&s= ect;23 chromosomes from father
§22 + X
or
&= sect;22 + Y
------=_NextPart_01C5C1F7.92B1B5D0 Content-Location: file:///C:/751B2C69/nickdennis240905(b)_files/slide0019.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Klinefelter syndrome: a clinical geneticist’s view
§A set of 23 chromosomes carries about <= /span>20,000 genes
§So our cells contain 2 copies of all th= ese genes
<= span class=3DBB style=3D'position:absolute;left:-4.31%'>§EXCEPT<= /b> most of the genes on the X chromosome in males, as most of the= m are not present on the Y chromosome
------=_NextPart_01C5C1F7.92B1B5D0 Content-Location: file:///C:/751B2C69/nickdennis240905(b)_files/slide0021.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Klinefelter syndrome: a clinical geneticist’s view
What do genes do?
§Most of them tell the cells how to make= a specific protein
§Proteins are the nuts and bolts and motors that make our bodies work
§For every protein we need to make, we <= /span>need at least one gene to tell us how to make it
------=_NextPart_01C5C1F7.92B1B5D0 Content-Location: file:///C:/751B2C69/nickdennis240905(b)_files/slide0005.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Klinefelter syndrome: a clinical geneticist’s view
Chromosomes of a male with Down Syndrome
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A small chromosome deletion- wi= th seve= re consequences
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Chromosomes of KS
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Content-Type: text/html; charset="us-ascii" Klinefelter syndrome: a clinical geneticist’s view
The X chromosome
<= span class=3DBB style=3D'position:absolute;left:-4.45%'>§Contains a= bout 5.3% of all the DNA
<= span class=3DBB style=3D'position:absolute;left:-4.52%'>§Contains a= bout 4% of all the genes
<= span class=3DBB style=3D'position:absolute;left:-4.56%'>§In contras= t, chromosome 21
<= span class=3DB1B style=3D'position:absolute;left:-4.1%'>§Contains o= nly 1.6% of the DNA
<= span class=3DB1B style=3D'position:absolute;left:-4.1%'>§And 1.2% o= f the genes
------=_NextPart_01C5C1F7.92B1B5D0 Content-Location: file:///C:/751B2C69/nickdennis240905(b)_files/slide0009.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Klinefelter syndrome: a clinical geneticist’s view
§So why does having an extra chromosome this size lead to Down syndrome
§
§
§
§And having an extra chromosome this size lead to the much less obvious KS?
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§Normal females have t= wo X chromosomes, but they switch off most of the genes on one or other of the= m
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§Males with Klinefelter syndrome do the same
XXY
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§So why are they any different from 46,XY males?
§Well, not all the genes on the “spare” X chromosome are switched off
<= span class=3DBB style=3D'position:absolute;left:-4.62%'>§And some o= f these genes are also present on the Y chromosome