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Hôpital-Camfrout, France

Jday-Daly I.,Laboratoire Of Biochimie Generale | Augereau-Vacher C.,Laboratoire Of Biochimie | De Curraize C.,Laboratoire Of Biochimie Metabolique | Fonfrede M.,Laboratoire Of Biochimie Metabolique | And 3 more authors.
Annales de Biologie Clinique | Year: 2011

As part of a tender AP-HP Paris Hospitals, an assessment of the reliability record of five blood glucose monitoring systems (BGMSs) (Optium Xceed (Abbott), Contour TS (Bayer), One Touch Ultra (Lifescan), Stat Strip Xpress (Nova) and Accu Check (Roche) and an evaluation of their sensitivity to changes in hematocrit were conducted in 4 hospitals of Paris. In terms of inaccuracy, all BGMSs have submitted CV repetability under the limits of acceptability. One BGMS (Lifescan) presented a CV of reproducibility outside limit of acceptability (13.1%). The inaccuracy was measured by a comparison method on multiparameter analyser relative to the hexokinase method for two sites, the glucose oxidase for the two others. The coefficients of correlation varied from 0.8405 to 0.9303. However, according to both defined acceptability criteria (absolute value difference between the result acquired on analyzer and those determined with the BGMS), the percentage of results outside acceptability was above 20% for two BGMSs (Abbott and Lifescan). Similarly, a net effect of changes in hematocrit was observed on the results of those two BGMSs.BGMSNovawas the most reliable, because of the correction device for hematocrit and blank substractions owed to interferences. In terms of expertise, BGMSs Nova and Roche have been selected with the best analytical performance and practicability satisfactory. In the future, accreditation with standard NF/EN 22870 requested for point of care testing, will require a close collaboration between biologists and clinicians to establish a system of strict quality control to detect deviations of these BGMSs.

Brassier A.,University of Paris Descartes | Boyer O.,University of Paris Descartes | Valayannopoulos V.,University of Paris Descartes | Ottolenghi C.,University of Paris Descartes | And 17 more authors.
Molecular Genetics and Metabolism | Year: 2013

Introduction: Patients with methylmalonic acidemia (MMA) may develop many complications despite medical treatment, in particular, severe central nervous system damage and chronic kidney disease (CKD). A kidney transplant may partially correct the metabolic dysfunctions. Liver, kidney and combined liver-kidney transplantations have been advocated but no guidelines are available to identify the most suitable organ to transplant. Patients and methods: Four patients with MMA (mut° phenotype) received a kidney graft because of repeated metabolic decompensations, with progression to CKD in 3 patients (end-stage kidney disease in two patients and CKD stage III in one patient with an estimated glomerular filtration rate [eGFR] of 40ml/min/1.73m2) but normal renal function in one (eGFR of 93ml/min/1.73m2) before transplantation. Results: The medium age at transplantation was 7.9. y (5-10.2) and the median follow-up was 2.8. years (1.8-4.6). Renal transplantation improved the relevant metabolic parameters in 4/4 patients and renal function in the patients with CKD. Plasma and urinary MMA levels immediately decreased and remained normal or subnormal (mean values of plasma MMA before transplantation 1530. μmol/L versus 240. μmol/L after transplantation, and mean values of urine MMA before transplantation 4700. mmol/mol creatinine versus 2300. mmol/mol creatinine after transplantation). No further acute metabolic decompensation was observed and protein-intake was increased from 0.60 to 0.83. g/Kg/day. One patient transplanted at age 9.7. years developed a hepatoblastoma at age 11. years with subsequent neurological complications and eventually died. The three other patients are alive. Two of them remained neurologically stable. The 3rd patient who displayed choreoathetosis transiently improved his neurological condition immediately after transplantation and then remained stable. Conclusion: Kidney transplantation represents an interesting alternative therapeutic option in methylmalonic aciduria, for renal complications but also as a "cellular therapy" that may significantly reduce metabolic decompensations and hospitalizations. However, further neurological impairment remains possible. © 2013.

Perdiz D.,University Paris - Sud | Mackeh R.,University Paris - Sud | Pous C.,University Paris - Sud | Pous C.,Laboratoire Of Biochimie Hormonologie | Baillet A.,University Paris - Sud
Cellular Signalling | Year: 2011

Microtubules are highly dynamic polymers of α/β tubulin heterodimers that play key roles in cell division and in organizing cell cytoplasm. Although they have been discovered more than two decades ago, tubulin post-translational modifications recently gained a new interest as their role was increasingly highlighted in neuron differentiation and neurodegenerative disorders. Here, we specifically focus on tubulin acetylation from its discovery to recent studies that provide new insights into how it is regulated in health and disease and how it impacts microtubule functions. Even though new mechanisms involving tubulin acetylation are regularly being uncovered, the molecular links between its location inside the microtubule lumen and its regulators and effectors is still poorly understood. This review highlights the emerging roles of tubulin acetylation in multiple cellular functions, ranging from cell motility, cell cycle progression or cell differentiation to intracellular trafficking and signalling. It also points out that tubulin acetylation should no longer be seen as a passive marker of microtubule stability, but as a broad regulator of microtubule functions. © 2010 Elsevier Inc.

Kaminsky P.,Nancy University Hospital Center | Acquaviva-Bourdain C.,Lyon University Hospital Center | Jonas J.,Nancy University Hospital Center | Pruna L.,Nancy University Hospital Center | And 4 more authors.
Muscle and Nerve | Year: 2011

Introduction: Multiple acyl-coenzyme A dehydrogenase deficiency (MADD), also called glutaric aciduria type II, is an inherited metabolic disorder resulting from a deficiency in electron transfer flavoprotein (ETF) or of its ubiquinone oxidoreductase (ETF-QO). It usually occurs in the neonatal period or in early infancy and, very rarely, in adolescents and young adult patients. Methods: We report the case of a 55-year-old woman who developed a painful subacute myopathy. Results: Lipid accumulation was found at biopsy. MADD was confirmed by plasma acylcarnitine profile and by assessment of ETF-QO activity in muscle. Conclusions: This study demonstrates that metabolic myopathies usually found in infancy may be also diagnosed in older patients. MADD may be easily treated by riboflavin and coenzyme Q10 and therefore should be included in the differential diagnosis of adult-onset painful myopathy. © 2011 Wiley Periodicals, Inc.

Froidevaux-Klipfel L.,University Paris - Sud | Poirier F.,University Paris - Sud | Boursier C.,University Paris - Sud | Crepin R.,University Paris - Sud | And 4 more authors.
Proteomics | Year: 2011

Cell resistance to low doses of paclitaxel (Taxol) involves a modulation of microtubule (MT) dynamics. We applied a proteomic approach based on 2-DE coupled with MS to identify changes in the MT environment of Taxol-resistant breast cancer cells. Having established a proteomic pattern of the microtubular proteins extracted from MDA-MB-231 cells, we verified by Western blotting that in resistant cells, α- and β-tubulins (more specifically the βIII and βIV isotypes) increased. Interestingly, four septins (SEPT2, 8, 9 and 11), which are GTPases involved in cytokinesis and in MT/actin cytoskeleton organization, were overexpressed and enriched in the MT environment of Taxol-resistant cells compared to their sensitive counterpart. Changes in the MT proteome of resistant cells also comprised increased kinesin-1 heavy chain expression and recruitment on MTs while dynein light chain-1 was downregulated. Modulation of motor protein recruitment around MTs might reflect their important role in controlling MT dynamics via the organization of signaling pathways. The identification of proteins previously unknown to be linked to taxane-resistance could also be valuable to identify new biological markers of resistance. © 2011 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

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