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ATP-binding cassette sub-family C member 6 (ABCC6)

Target
ABCC6
Molecular classification
Transporter, ATP-binding cassette transporter family (ABC transporter), Multidrug resistance-associated protein (MRP subfamily)
01

Overview

ATP-binding cassette sub-family C member 6 (ABCC6) is a transmembrane protein predominantly expressed in the liver and kidney, where it acts as an ATP-dependent efflux transporter for organic anions and possibly other yet unidentified substrates[2][4][7][9]. ABCC6 is a member of the ABC transporter superfamily, specifically the MRP (multidrug resistance-associated protein) subfamily that actively transports molecules across cell membranes using ATP hydrolysis[1][3][4]. Its physiological function includes regulation of extracellular nucleotide levels—particularly via ATP release and subsequent pyrophosphate production—playing a critical role in mineralization homeostasis. Pathogenic variants of ABCC6 cause pseudoxanthoma elasticum (PXE), a heritable disorder characterized by abnormal calcification of connective tissues, and are also associated with early-onset atherosclerosis[2][4][5]. ABCC6 has no well-established direct pharmacological inhibitors or activators in current medical use, but its role in drug resistance and substrate efflux may have clinical implications.

Other names
ABCC6Multidrug resistance-associated protein 6 (MRP6)Multi-specific organic anion transporter-E (MOAT-E)Anthracycline resistance-associated protein (ARA)ABC34EST349056MLP1PXEPXE1URG7
02

Mechanism of action

Drugs/compounds that modulate ABCC6 function would likely affect cellular efflux of anionic substrates or modulate systemic levels of pyrophosphate and nucleotides, influencing mineralization and drug resistance phenotypes[1][4][7].

03

Biological functions

Extrusion of physiological compounds and xenobiotics from cellsOrganic anion efflux pumpRegulation of cellular nucleotide (e.g., ATP) releasePrevention of ectopic mineralization via modulation of extracellular pyrophosphate levels
04

Disease associations

Pseudoxanthoma elasticum (PXE)Premature atherosclerosisPotential roles in other ectopic mineralization disorders
05

Safety considerations

Loss-of-function or genetic variants lead to ectopic calcification (PXE, vascular calcification)Systemic effects due to altered pyrophosphate metabolismPotential impact on drug pharmacokinetics (due to transporter function, theorized but not confirmed for clinical management)
06

Interacting drugs

No specific widely used therapeutic drugs shown to directly target ABCC6 in the given sources. Some literature suggests possible interaction with anthracyclines and other drugs due to its transporter role, but these are not FDA-approved ABCC6-specific drugs[6][4].
07

Biomarkers

ABCC6 mutations (e.g., R1141X, 23-29del) serve as biomarkers for pseudoxanthoma elasticum[2][4].Pyrophosphate levels as a functional readout for ABCC6 activity may act as a biomarker in PXE.

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