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AVANCES EN LA GENÉTICA Y LA ETIOPATOGENIA DE LAS DEMENCIAS = = FRONTOTEMPORALES<= /div>
Mª Elena Toribio Díaz, Jaume Morera Guitart,= Susana Palao Duarte.
UNIDAD DE NEUROLOGÍA DE LA CONDUCTA Y DEMENCIAS <= /b>(CDP-ALZ). Hospital San Vicente. = San Vicente del Raspeig. Alicante
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EPIDEMIOLOGÍA
Tercera= causa de demencia degenerativa después de la Enfermedad de Alzheimer y de la demencia con cuerpos de Lewy
Segunda= causa de demencia en menores de 65 años después de la Enfermedad de Alzheimer
–<= /span>20% de las demencias preseniles
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CLÍNICA
NEUROPATOLOGÍA
HERENCIA
GENÉTICA
HETEROG= ENEIDAD
DEMEN= CIAS
FRONT= OTEMPORALES
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CLÍNICA
NEUROPATOLOGIA
GENÉTICA
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El diagnóstico neuropatológico= de las enfermedades neurodegenerativas se basa en la identificación de acúmulos, neuronales o no, consti= tuídos por <= i>proteínas agregadas:
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El diagnóst= ico neuropatológico de las = enfermedades neurodegenerativas se basa en la identificación de acúmulos, neuronales o no, constitu= ídos por <= i>proteínas agregadas:
= Ø Depósito amiloide â Enf de Alzheimer
ØProte&iacut= e;na Tau  â DFT – PSP – DCB – Enf Alzh = ;
Øa- Sinucleína â a- sinucleopatías
= Ø  Proteínas Priónicas&= nbsp; â Enf Creutzfeld-Jacob&= #13;
ØPoliglutami= nas  â Enf Huntington
ØUbiquitina = &ac= irc;DFT
ØNeurofilame= ntos &ac= irc;DFT
ØOtras <= /span>
Ø
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El diagnóst= ico neuropatológico de las enfermedades neurodegenerativas se basa en la identificación de acúmulos= , neuronales o no, constituídos por = proteína= s agregadas:
= Ø Depósito amiloide â Enf de Alzheimer
ØProte&iacut= e;na Tau  â DFT – PSP – DCB – Enf Alzh = ;
Øa- Sinucleína â a- sinucleopatías
= Ø  Proteínas Priónicas&= nbsp; â Enf Creutzfeld-Jacob&= #13;
ØPoliglutami= nas  â Enf Huntington
ØUbiquitina = &ac= irc;DFT
ØNeurofilame= ntos &ac= irc;DFT
ØOtras <= /span>
Ø ME= CANISMO PATOGENÉTICO
â ACÚMULO DE PROTEÍNA ANÓMALA
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 Proteina anormal
 Disfunción Neuronal
(selectiva)
 Activa Apoptosis
 Muerte Neuronal
(en el tiempo)
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DEMENCIAS FRONTOTEMPORALES
ACÚMULO DE PROTEÍNA TAU
NO SIEMPRE
ACÚMULO DE PROTEÍNA ANÓMALA
El obj= etivo de la presentación es poner de manifiesto que en las demencias frontotemporales no siempre existe un acúmulo de proteína t= au (anómala) y que no siempre el acúmulo de una proteína anómala es el mecanismo etiopatogénico subyacente.
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El cit= oesqueleto celular está constituído por microfilamentos, microtúbulos y filamentos intermedios. La proteína tau se u= ne a los microtúbulos favoreciendo su ensamblaje y estabilizació= n, contribuyendo así al mantenimiento de la estructura celular.
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