Porphyrin Metabolism and Disorders

Isolation and In vitro Characterization of BchE, the Cobalamin-Dependent Anaerobic Magnesium Protoporphyrin IX Monomethylester Cyclase Involved in Bacteriochlorophyll Biosynthesis

This paper isolates and characterizes BchE, a cobalamin-dependent radical SAM enzyme, enabling in vitro studies of its role in bacteriochlorophyll biosynthesis.

An adaptor for feedback regulation of heme biosynthesis by the mitochondrial protease CLPXP

The paper identifies POLDIP2 as a heme-sensing adaptor that facilitates CLPXP-mediated degradation of ALAS, elucidating the mechanism of heme-induced feedback regulation in heme biosynthesis.

Evolutionarily divergent DUF4465 domains have a common vitamin B12-binding function

The paper identifies B12-binding function in multiple DUF4465 proteins across diverse bacterial species.

A reversible feedback mechanism regulating mitochondrial heme synthesis

This study identifies a reversible feedback mechanism where heme inhibits mature mitochondrial ALAS2, regulating heme synthesis and providing insights into its spatial control.

Microbes display broad diversity in cobamide preferences

The paper characterizes bacterial cobamide preferences by testing growth under specific cobamide-dependent conditions, revealing broad diversity in cobamide usage across organisms and metabolic pathways.

Systematically testing human HMBS missense variants to reveal mechanism and pathogenic variation

This study systematically tests HMBS missense variants to determine their functional impact and distinguish pathogenic from benign variants.