Identificata proteina che contrasta progressione Sla

9 novembre 2020 – La proteina CXCL13, appartenente alla famiglia delle chemochine, che viene attivata dai neuroni che comandano i movimenti muscolari (motoneuroni), ha effetti benefici contro la progressione della sclerosi laterale amiotrofica (Sla) in un modello animale. Lo hanno scoperto i ricercatori dell’ Istituto Mario Negri di Milano, secondo cui potrebbe essere usata come marcatore per la discriminazione precoce della malattia rispetto ad altri disturbi neurologici, come la sclerosi multipla.
Questo potrebbe essere molto importante perchè la diagnosi rapida della Sla “potrebbe essere fondamentale per un trattamento efficace”, commenta Giovanni Nardo, principale ricercatore dello studio, pubblicato on line sulla rivista scientifica EBioMedicine.
I ricercatori spiegano che evidenze preliminari avevano messo in luce un significativo incremento di CXCL13 nei topi con Sla, cosa che poteva suggerire una correlazione tra l’attivazione della chemochina e una progressione della malattia più rapida.
Ma la neutralizzazione della chemochina in topi Sla ha invece portato a un peggioramento della malattia, suggerendo, al contrario, un effetto benefico della presenza di CXCL13 nella malattia.
“L’aspetto rilevante è che durante la malattia – dice Maria Chiara Trolese, co-investigatore e primo autore del lavoro – i motoneuroni esprimono alti livelli di questa chemochina. La specifica soppressione di CXCL13 ha quindi privato le cellule di un processo di protezione precedentemente ignoto. Infatti, abbiamo osservato che il silenziamento di CXCL13 induce una perdita di motoneuroni e un incremento dell’infiammazione, mentre la sua somministrazione preserva i motoneuroni dalla degenerazione”.
Coerentemente con l’azione protettiva di CXCL13 nel modello animale, i livelli di CXCL13 sono stati osservati aumentati anche nei motoneuroni spinali rimasti dei pazienti Sla mentre sono significativamente ridotti nel liquido cerebrospinale rispetto ai pazienti con sintomi non neurologici o con sclerosi multipla.
Cosa che fa dire a Caterina Bendotti, capo del laboratorio di Neurobiologia Molecolare, che “i livelli ridotti di CXCL13 nel liquido cerebrospinale dei pazienti di Sla potrebbero essere un indice della degenerazione dei motoneuroni, mettendo in luce questa chemochina come marcatore clinico per la discriminazione precoce della malattia rispetto a disturbi neurologici con elevata componente infiammatoria come la polineuropatia cronica demielinizzante e la sclerosi multipla”.

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