home libri books Fumetti ebook dvd top ten sconti 0 Carrello


Torna Indietro
ARGOMENTO:  BOOKS > BIOLOGIA > BIOLOGIA > NEUROBIOLOGIA

nóbrega clévio (curatore); pereira de almeida luís (curatore) - polyglutamine disorders

Polyglutamine Disorders

;




Disponibilità: Normalmente disponibile in 15 giorni


PREZZO
140,98 €
NICEPRICE
133,93 €
SCONTO
5%



Questo prodotto usufruisce delle SPEDIZIONI GRATIS
selezionando l'opzione Corriere Veloce in fase di ordine.


Pagabile anche con Carta della cultura giovani e del merito, 18App Bonus Cultura e Carta del Docente


Facebook Twitter Aggiungi commento


Spese Gratis

Dettagli

Genere:Libro
Lingua: Inglese
Editore:

Springer

Pubblicazione: 06/2019
Edizione: Softcover reprint of the original 1st ed. 2018





Trama

This book provides a cutting-edge review of polyglutamine disorders. It primarily focuses on two main aspects: (1) the mechanisms underlying the pathologies’ development and progression, and (2) the therapeutic strategies that are currently being explored to stop or delay disease progression. 

Polyglutamine (polyQ) disorders are a group of inherited neurodegenerative diseases with a fatal outcome that are caused by an abnormal expansion of a coding trinucleotide repeat (CAG), which is then translated in an abnormal protein with an elongated glutamine tract (Q). To date, nine polyQ disorders have been identified and described: dentatorubral-pallidoluysian atrophy (DRPLA); Huntington’s disease (HD); spinal–bulbar muscular atrophy (SBMA); and six spinocerebellar ataxias (SCA 1, 2, 3, 6, 7, and 17).

The genetic basis of polyQ disorders is well established and described, and despite important advances that have opened up the possibility of generating genetic models of the disease, the mechanisms that cause neuronal degeneration are still largely unknown and there is currently no treatment available for these disorders. Further, it is believed that the different polyQ may share some mechanisms and pathways contributing to neurodegeneration and disease progression.






Sommario

Clinical features of Huntington’s disease.- Genetic rodent models of Huntington’s disease.- Mitochondrial dysfunction in Huntington’s disease.- RNA related pathology in Huntington’s disease.- Spinal and bulbar muscular atrophy: from clinical genetic features and molecular pathology to mechanisms underlying disease toxicity.- Spinocerebellar Ataxia Type 1: Molecular Mechanisms Of Neurodegeneration And Preclinical Studies.- Spinocerebellar Ataxia, type 6.- Spinocerebellar ataxia type 2.- Molecular mechanisms and therapeutic strategies in Spinocerebellar ataxia type 7.- Spinocerebellar ataxia type 17 (SCA17).- The neuropatholy of spinocerebellar ataxia type 3/Machado-Joseph disease.- Origins and spread of Machado-Joseph disease ancestral mutational events.- Clinical features in Machado-Joseph disease.- Polyglutamine-independent features in ataxin-3 aggregation and pathogenesis of Machado-Joseph disease.- Animal models in Machado-Joseph disease.- Towards the identification of molecular biomarkers of Spinocerebellar ataxia type 3/Machado-Joseph disease.- Planning future clinical trials for Machado Joseph Disease.- Molecular mechanisms and cellular pathways implicated in Machado-Joseph disease pathogenesis.- Pharmacological therapies for Machado-Joseph disease.- Gene therapy for Polyglutamine diseases.- Stem cell-based therapies for Polyglutamine diseases.




Autore

Clevio Nobrega Ph.D. and Luis Pereira de Almeida Ph.D.
University of Coimbra, Center for Neurosciences and Cell Biology, FMUC, Coimbra, Portugal











Altre Informazioni

ISBN:

9783319891033

Condizione: Nuovo
Collana: Advances in Experimental Medicine and Biology
Dimensioni: 235 x 155 mm Ø 730 gr
Formato: Brossura
Illustration Notes:VIII, 469 p. 41 illus., 39 illus. in color.
Pagine Arabe: 469
Pagine Romane: viii


Dicono di noi