Disruption of Hox9,10,11 function results in cellular level lineage infidelity in the kidney

Keri A. Drake, Mike Adam, Robert Mahoney, S. Steven Potter

Research output: Contribution to journalArticlepeer-review

10 Scopus citations


Hox genes are important regulators of development. The 39 mammalian Hox genes have considerable functional overlap, greatly confounding their study. In this report, we generated mice with multiple combinations of paralogous and flanking Abd-B Hox gene mutations to investigate functional redundancies in kidney development. The resulting mice developed a number of kidney abnormalities, including hypoplasia, agenesis, and severe cysts, with distinct Hox functions observed in early metanephric kidney formation and nephron progenitor maintenance. Most surprising, however, was that extensive removal of Hox shared function in these kidneys resulted in cellular level lineage infidelity. Strikingly, mutant nephron tubules consisted of intermixed cells with proximal tubule, loop of Henle, and collecting duct identities, with some single cells expressing markers associated with more than one nephron segment. These results indicate that Hox genes are required for proper lineage selection/maintenance and full repression of genes involved in cell fate restriction in the developing kidney.

Original languageEnglish (US)
Article number6306
JournalScientific reports
Issue number1
StatePublished - Dec 1 2018

ASJC Scopus subject areas

  • General


Dive into the research topics of 'Disruption of Hox9,10,11 function results in cellular level lineage infidelity in the kidney'. Together they form a unique fingerprint.

Cite this