paperKB
coga / coga-kb
Help
Sign in

Chunk #49 — BAF complexes in neurological disorders — Nicolaides-Baraitser syndrome

Source
The role of BAF (mSWI/SNF) complexes in mammalian neural development.
Embedded
yes

Text

ATPase domain, particularly in the ATP-binding region and the helicase region; this suggests that these missense mutations alter the activity of the ATPase and lead to gain-of-function or dominant negative effects. Given the overall patterns of subunit mutations in CSS and NCBRS, the BAF47 mutation in one NCBRS patient is unusual and may reflect the extensive overlap of clinical features between the two disorders.