Menkes Disease

Dear Michael Petris,

In Maternofetal and neonatal copper requirements revealed by enterocyte-specific deletion of the Menkes disease protein (of which you are the corresponding author), it was noted that the infant mice were specifically lacking Atp7a protein within intestinal enterocytes, and that supplementating copper in lactating females or intravenous copper injections of the induced Menkes offspring extended their lifespan.

My question is, could transplanting inrestinal lining save these rats, and thus also children with Menkes disease?

Deborah Barges

Shanbhag V, Jasmer-McDonald K, Zhu S, Martin AL, Gudekar N, Khan A, Ladomersky E, Singh K, Weisman GA, Petris MJ. ATP7A delivers copper to the lysyl oxidase family of enzymes and promotes tumorigenesis and metastasis. Proc Natl Acad Sci U S A. 2019 Apr 2;116(14):6836-6841. doi: 10.1073/pnas.1817473116. Epub 2019 Mar 19. PMID: 30890638; PMCID: PMC6452744.

©Deborah Barges Dec 2020, open access