Author

Lawton F. Long

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Sep 2026

A yeast model for the functional analysis of SRP54 mutations associated with severe congenital neutropenia.

Mutations in the human SRP54 gene are linked to the pathophysiology of severe congenital neutropenia (SCN). SRP54 is a key protein comprising one of the six protein subunits of the signal recognition particle responsible for cotranslational targeting of proteins to the ER. Crystal structures and biochemical characteriz...

Lawton F. Long, Shivani Biskunda, Ming Yang et al. · The FEBS Journal · 0 citations

#protein folding Open access Oct 2026

Structure and Function of Archaeal SRP54 Protein Mutants Associated with Human Severe Congenital Neutropenia

SRP54, a key component of the signal recognition particle, is a highly conserved enzyme both structurally and functionally. The protein plays a vital role in cellular protein translocation by interacting with the translating ribosome and a membrane receptor, thereby aiding protein folding and trafficking. Recent stud...

Ming Yang, Andrew D. Brim, Lawton F. Long et al. · ACS Omega · 0 citations