[HTML][HTML] ATP-dependent mitochondrial porphyrin importer ABCB6 protects against phenylhydrazine toxicity

DL Ulrich, J Lynch, Y Wang, Y Fukuda… - Journal of Biological …, 2012 - ASBMB
Abcb6 is a mammalian mitochondrial ATP-binding cassette (ABC) transporter that regulates
de novo porphyrin synthesis. In previous studies, haploinsufficient (Abcb6+/−) embryonic
stem cells showed impaired porphyrin synthesis. Unexpectedly, Abcb6−/− mice derived from
these stem cells appeared phenotypically normal. We hypothesized that other ATP-
dependent and/or-independent mechanisms conserve porphyrins. Here, we demonstrate
that Abcb6−/− mice lack mitochondrial ATP-driven import of coproporphyrin III. Gene …