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Clin Transl Sci ; 6(6): 485-6, 2013 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-24330694

RESUMEN

Pseudoxanthoma elasticum (PXE), caused by mutations in the ABCC6 gene, demonstrates progressive build-up of calcium phosphate and proteoglycans in the skin, eye, and arteries, and is associated to myocardial infarctions, stroke, blindness, and elevated carotid intima-media thickness (CIMT). Although CIMT reduction with magnesium (Mg) has been documented in a mouse model for PXE (Abcc6(-/-) ), it is not clear if Mg is effective in humans with PXE to reduce CIMT. To examine this, we calculated the rate of change of CIMT (washout) in 15- and 12-month-old Abcc6(-/-) mice fed standard rodent diet with or without Mg supplementation for 2 months. Using means in untreated 15- and 12-month-old Abcc6(-/-) mice (145 and 120 µm, respectively), the rate of change was 8.3 µm/month. Using means in treated 15- and 12-month-old Abcc6(-/-) mice (118 and 104.6 µm, respectively), the rate of change was 4.5 µm. Compared to normal progression of CIMT in humans without PXE, PXE has advanced atherosclerosis and possibly a higher CIMT rate of change. This experiment may portend, at least in PXE, the rationale for a 1-year oral Mg CIMT clinical trial and may be useful for application in other progressive mineralizing disorders like atherosclerosis.


Asunto(s)
Transportadoras de Casetes de Unión a ATP/deficiencia , Arterias Carótidas/efectos de los fármacos , Enfermedades de las Arterias Carótidas/tratamiento farmacológico , Grosor Intima-Media Carotídeo , Óxido de Magnesio/farmacología , Seudoxantoma Elástico/tratamiento farmacológico , Transportadoras de Casetes de Unión a ATP/genética , Animales , Arterias Carótidas/metabolismo , Arterias Carótidas/patología , Enfermedades de las Arterias Carótidas/genética , Enfermedades de las Arterias Carótidas/metabolismo , Enfermedades de las Arterias Carótidas/patología , Modelos Animales de Enfermedad , Ratones , Ratones Noqueados , Proteínas Asociadas a Resistencia a Múltiples Medicamentos , Seudoxantoma Elástico/genética , Seudoxantoma Elástico/metabolismo , Seudoxantoma Elástico/patología , Factores de Tiempo
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