We open HMG in August!
Tuesday, 30 July 2013 00:00
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Cover: Proposed molecular mechanism for mitochondrial impairment in X-linked adrenoleukodystrophy. As C26:0 cannot enter peroxisomes for degradation due to the loss of the peroxisomal transporter ABCD1, it accumulates intracellularly affecting the inner mitochondrial membrane impermeability by unknown mechanisms. This alteration may allow a proton flux into the matrix decreasing Δψm, together with a certain extent of electron leakage promoting ROS formation. These ROS oxidise mitochondrial proteins of TCA cycle and OXPHOS, leading to impaired bioenergetics and respiration, and also oxidise mtDNA, contributing to a devil cycle of mitochondrial dysfunction and ultimately, cell demise. See article by López-Erauskin et al., pp. 3296–3305.
Last Updated on Tuesday, 30 July 2013 09:02
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La ciclofilina D se perfila como nueva diana en adrenoleucodistrofia ligada al X
Thursday, 28 February 2013 00:00
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From Diario Médico
February 28, 2013
Javier Granada
Dos trabajos publicados en la revista Brain por un equipo del Idibell, de Barcelona, apuntan a una nueva diana que parece revertir síntomas asociados a la adrenoleucodistrofia ligada al cromosoma X. Derivada del estudio de los fibroblastos, la ciclofilina D parece revertir síntomas ligados a la adrenoleucodistrofia ligada al cromosoma X (X-ALD) (ver el artículo). El equipo de Aurora Pujol ha publicado este mismo lunes un segundo trabajo. En él se describe que el inmunoproteasoma, además de responsable de los procesos inflamatorios, se activa con el estrés oxidativo crónico (ver el artículo).
Last Updated on Tuesday, 30 July 2013 09:04
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Gene Therapy Halts Fatal Brain Disease
Tuesday, 10 November 2009 00:00
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From Wired Science
November 5, 2009
By Brandon Keim
Scientists have used gene therapy to halt the progression of adrenoleukodystrophy, a fatal neurodegenerative disease caused by a single defective gene, in two seven-year-old boys.
It took more than a decade to refine the therapy, in which stem cells taken from the boys’ bone marrow were hacked with healthy copies of the gene, then returned to their bodies. Without them, the boys would soon be dead.
Last Updated on Tuesday, 10 November 2009 12:02
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Un cóctel de antioxidantes, potencial terapia para la adrenoleucodistrofia
Thursday, 10 February 2011 00:00
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From Diario Médico
February 3, 2011
Un grupo dirigido por Aurora Pujol, del Instituto de Investigación Biomédica de Bellvitge (Idibell), ha descubierto que la combinación de tres antioxidantes revierte los principales síntomas de la adrenoleucodistrofia ligada al cromosoma X. El ensayo piloto en humanos está en fase de reclutamiento y pendiente de aprobación para comenzar.
Last Updated on Friday, 11 February 2011 12:08
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Gene therapy could cure Lorenzo’s Oil brain disease
Tuesday, 10 November 2009 00:00
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From The Times
November 6, 2009
A devastating brain disease that kills boys by the time of adolescence has been stopped in its tracks by a gene therapy that raises hope for thousands of families.
The treatment has halted the development of adrenoleukodystrophy (ALD) — the genetic disorder featured in the film Lorenzo’s Oil — in two Spanish boys aged 7 who would otherwise have suffered progressive and ultimately fatal brain damage. A third boy has also been treated, but too recently for doctors to know whether it has been successful.
Last Updated on Tuesday, 10 November 2009 11:56
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