Biología Celular TAU

Filamentous tauopathies protein and/or microtubule defects are also associated with a range of neurodegenerative disorders known as tauopathies. Tauopathies are generally characterized by the presence of NFT and the absence of neuritic plaques. As with AD, NFT comprise either of neuritic plaques. As with AD, NFT comprise either

mutated tau (in the case of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP17)), or

hyperphosphorylated tau protein. In addition to AD,

recent evidence indicates that NFT are associated with Picks's Disease, FTDP17, cortico-basal degeneration, progressive supranuclearpalsy, and amyotropic lateral

sclerosis/parkinsonism-dementia complex of Guam, among others. as it stands however, the sole set of know mutations in tauophaties are ssociated with FTDP12, in the C-terminal portion of tauopathies, three imperfecto repeats are present and each contains a binging domain to microtubules. several mutations in exon 10 alter the ratio og microtubule binding domains from trhee to four repeats. the four repeat from oh tauopathies binds more strongly to microtubules and tends to aggregate more rapidly than the three-repeat form.

NFT may resul from polymerization of free tauopathies subnits thus depleting the amount of tauopathies avaiabe for binging. mice null for a tauopathies shows little phenotupe while mice over expressing the four-repeat form of tauopathies display axon adnormalities but no NFT. these data indicate that severe effects on microtubule stability are unlikely to account for disease. however, subtle differences in stability are unlikelu to account for disease. however, subtle differeces in stabilization of microtubules may play an important and common component for neuronal survival over a lifeime.

Proteína filamentosa tauopatías y / o defectos de microtúbulos también se asocian con una amplia gama de trastornos neurodegenerativos conocidos como tauopatías . Tauopatías se caracterizan generalmente por la presencia de NFT y la ausencia de placas neuríticas . Al igual que con AD , NFT comprenden cualquiera de las placas neuríticas . Al igual que con AD , NFT comprenden bien

tau mutada ( en el caso de la demencia frontotemporal y parkinsonismo ligado al cromosoma 17 ( FTDP17 ) ) , o

proteína tau hiperfosforilada . Además de AD ,

la evidencia reciente indica que NFT están asociados con la enfermedad de Picks , FTDP17 , degeneración cortico -basal , supranuclearpalsy progresiva y lateral amiotrófica

esclerosis / parkinsonismo - demencia compleja de Guam , entre otros . tal y como está sin embargo , el único conjunto de sabe mutaciones en tauophaties son ssociated con FTDP12 , en la porción C -terminal de tauopatías , tres imperfecto repite están presentes y cada uno contiene un dominio de atracones a los microtúbulos . varias mutaciones en el exón 10 modificar la relación og dominios de unión

...