Demonstration of biophysical determinants impacting Osh6-mediated lipid transport in a model membrane system, revealing the subtle orchestration between the transporter, cargo, and membrane properties...

Carefully analyze the key features in this pediatric case — failure to thrive, steatorrhea, hypotonia, developmental delay, retinitis pigmentosa, low cholesterol, acanthocytosis, and lipid-laden enterocytes. Focus on the combination of gastrointestinal, neurological, ophthalmologic, and laboratory findings to determine the underlying molecular defect. Then, select the correct option from the provided choices.

We report the crystal structure of human PITPα in complex with microcolin H at 2.0 Å resolution, revealing that microcolins bind covalently within the lipid-binding cavity and stabilize PITPα in an open conformation with a markedly enlarged cavity relative to the lipid-bound state. These results define the structural basis of PITP inhibition by microcolins and underscore the importance of using ligand-bound conformations for accurate structure-based modeling.

Subcellular lipid composition and transport significantly influence the physiological and pathological functions of both cells and organelles. However, lipid transport and turnover between organelles remain poorly understood due to a lack of methods for selectively labeling lipids in organelles.

Abstract. ATP-binding cassette (ABC) transporters are essential membrane proteins that couple ATP hydrolysis to move diverse substrates across lipid bilayers through large-scale conformational changes...