Fidelity of the elongation step
during translation is crucial for
proper folding dynamics of nascent
proteins — a process regulated by the
Elongator complex. Several studies
have linked mutations in subunits
of the Elongator complex to various
neurodegenerative disorders.
This study sought to examine the
mechanism of Purkinje neuron
degeneration, which leads to
cerebellar ataxia. Using a mouse
model, the authors examined a
mutation in Elongator complex
subunit 6 and its role in the
degeneration of Purkinje neurons.
Reveal how a single point
mutation affects the
neuropathology of ataxia
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Molecular
interactions
studied
Targets: purified Elp456
Elongator Complexes
Ligands: Cy5-labeled tRNA
Ala
and tRNA
Cys
Method
MST using Monolith
nanoDSF using Prometheus
Using nanoDSF and MST, researchers
confirmed that this mutation
negatively affects the function and
stability of the Elongator complex,
interfering with the regulation of
translation. This data provides
novel mechanistic insight into the
development of cerebellar ataxia
caused by protein aggregation and
subsequent neuronal death.
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