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Background  ATP-binding cassette (ABC) transporters are involved in a huge range of physiological processes. Mutations in the  ABCC6  gene cause pseudoxanthoma elasticum, a metabolic disease with progressive soft tissue calcification.    Methods  The aim of the present study was to analyze gene expression levels of selected ABC transporters associated with cholesterol homeostasis in metabolic active tissues, such as the liver, kidney and white adipose tissue (WAT) of  Abcc6   −/−   mice from an early and late disease stage (six-month-old and 12-month-old mice).    Results  The strongest regulation of ABC transporter genes was observed in the liver tissue of six-month-old  Abcc6   −/−   mice. Here, we found a significant increase of mRNA expression levels of phospholipid, bile salt and cholesterol/sterol transporters  Abcb1b ,  Abcb11 ,  Abcg1, Abcg5  and  Abcg 8.  Abcd2  mRNA expression was increased by 3.2-fold in the liver tissue. We observed strong upregulation of  Abca3  and  Abca1  mRNA expression up to 3.3-fold in kidney and WAT, and a 2-fold increase of  Abca9  mRNA in the WAT of six-month-old  Abcc6  knockout mice. Gene expression levels of  Abcb1b  and  Abcg1  remained increased in the liver tissue after an age-related disease progression, while we observed lower mRNA expression of  Abca3  and  Abca9  in the kidney and WAT of 12-month-old  Abcc6   −/−   mice.    Conclusions  These data support previous findings that Abcc6 deficiency leads to an altered gene expression of other ABC transporters depending on the status of disease progression. The increased expression of fatty acid, bile salt and cholesterol/sterol transporters may be linked to an altered cholesterol and lipoprotein metabolism due to a loss of Abcc6 function.    Electronic supplementary material  The online version of this article (10.1186/s12944-018-0943-x) contains supplementary material, which is available to authorized users.

Abcc6 deficiency in mice leads to altered ABC transporter gene expression in metabolic active tissues