Molecular Classification
ABC Transporter Domain, Nucleotide-Binding Domain
Disease Roles
Cystic Fibrosis

Cystic Fibrosis Transmembrane Conductance Regulator Nucleotide-Binding Domain 1 Overview

The nucleotide-binding domain 1 (NBD1) of the cystic fibrosis transmembrane conductance regulator (CFTR) is a crucial component involved in channel activation and regulation. It binds and hydrolyzes ATP, driving conformational changes that influence chloride channel gating. The domain contains the common cystic fibrosis-causing mutation ΔF508. Proper assembly and function require dynamic interactions between both nucleotide-binding domains.

Mechanism of Action

Modulation of CFTR folding and function to improve chloride transport

Biological Functions

ATP Binding
ATP Hydrolysis
Regulation of Chloride Channel Gating

Disease Associations

Cystic Fibrosis

Safety Considerations

  • Potential for off-target effects due to similarity with other ABC transporters
  • Challenges in achieving sufficient correction of CFTR function in patients with severe mutations