Increasing Frataxin
ironj...@aol.com
iron on board.
The replacement erythropoietin in this study leads to an increase of
the frataxin levels.
One might wonder then would the erythropoietin levels rise in
Friedreich's ataxia BY .. phlebotomy / bloodletting / venesection /
iron reduction and then ALSO cause the frataxin to rise .. ?
----------------
Neurological effects of recombinant human erythropoietin in
Friedreich's ataxia: A clinical pilot trial.
Boesch S, Sturm B, Hering S, Scheiber-Mojdehkar B, Steinkellner H,
Goldenberg H, Poewe W
Mov Disord 2008 Aug 29.
In a "proof-of-concept" study, we demonstrated that recombinant human
erythropoietin (rhuEPO) increases frataxin levels in Friedreich's
ataxia (FRDA) patients. We now report a 6-month open-label clinical
pilot study of safety and efficacy of rhuEPO treatment in FRDA. Eight
adult FRDA patients received 2.000 IU rhuEPO thrice a week
subcutaneously. Clinical outcome measures included Ataxia Rating
Scales. Frataxin levels and indicators for oxidative stress were
assessed. Hematological parameters were monitored biweekly. Scores in
Ataxia Rating Scales such as FARS (P = 0.0063) and SARA (P = 0.0045)
improved significantly. Frataxin levels increased (P = 0.017) while
indicators of oxidative stress such as urine 8-OHdG (P = 0.012) and
peroxide levels decreased (P = 0.028). Increases in hematocrit
requiring phlebotomies occurred in 4 of 8 patients. In this
explorative open-label clinical pilot study, we found an evidence for
clinical improvement together with a persistent increase of frataxin
levels and a reduction of oxidative stress parameters in patients
with
FRDA receiving chronic treatment with rhuEPO. Safety monitoring with
regular blood cell counts and parameters of iron metabolism is a
potential limitation of this approach. (c) 2008 Movement Disorder
Society.
Movement disorders : official journal of the Movement Disorder
Society
[Mov Disord]
--------------------------------------------------------------------------------
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ironj...@aol.com
erythropoietin <<
One might wonder whether this iron chelation ALSO raises ..
erythropoietin and possibly frataxin.
"Reduced neuropathy and ataxic-gait in the youngest patients."
Blood. 2007 Mar 22;
Selective iron chelation in Friedreich ataxia. Biological and clinical
implications.
Boddaert N, Le Quan Sang KH, Rotig A, Leroy-Willig A, Gallet S,
Brunelle F, Sidi D, Thalabard JC, Munnich A, Cabantchik ZI.
Pediatric Radiology Unit, ERM0205, Hopital Necker-Enfants Malades &
Universite Paris V Rene Descartes, Paris, France.
Genetic disorders of iron metabolism and chronic inflammation often
evoke local iron accumulation.
In Friedreich-ataxia, decreased iron- sulphur-cluster and haem
formation leads to mitochondrial iron accumulation and ensuing
oxidative damage that affect primarily
sensory neurons, myocardium and endocrine glands.
We assessed the possibility of reducing brain iron accumulation in
Friedreich-ataxia patients with a membrane-permeant chelator capable
of shuttling
chelated-iron from cells to transferrin, using regimens suitable for
patients with no systemic iron overload.
Brain MRI of Friedreich- ataxia patients compared to age-matched
controls revealed smaller and irregularly shaped dentate-nuclei with
significantly (p<0.027) higher H-relaxation rates R2*, indicating
regional iron accumulation. A six-
month treatment with 20-30mg/kg/d deferiprone applied on 11/20
adolescent patients with no overt cardiomyopathy reduced R2* from
18.3+/-1.6 to 15.7+/-0.7msec(-1) (p<0.002) specifically in dentate
nuclei and proportionally to the initial R2* (r=0.90).
Chelator- treatment caused no apparent haematological or neurological
side-
effects, while reducing neuropathy and ataxic-gait in the youngest
patients.
To our knowledge, this is the first clinical demonstration of
chelation removing labile iron accumulated in a specific brain area
implicated in a neurodegenerative disease.
The use of moderate chelation for relocating iron from areas of
deposition to areas of deprivation has clinical implications for
various neurodegenerative and haematological disorders.
PMID: 17379741