The Generic Authentication Architecture (GAA) is a standardised extension to the mobile telephony security infrastructures that supports the provision of security services to network applications. We have proposed a generalised version of GAA which enables almost any pre-existing infrastructure to be used as the basis for the provision of generic security services, and have examined a GAA insta...

Purpose: A variety of cancers, including malignant gliomas, overexpress transforming growth factor-β (TGF-β), which helps tumors evade effective immune surveillance through a variety of mechanisms, including inhibition of CD8 CTLs and enhancing the generation of regulatory T (Treg) cells. We hypothesized that inhibition of TGF-β would improve the efficacy of vaccines targeting glioma-associated...

EDITOR—Friedreich’s ataxia (FRDA, MIM 229300) is an autosomal recessive, progressive, neurodegenerative disorder. It is the most common of all hereditary ataxias, with an estimated prevalence of 1 in 50 000, and a carrier frequency calculated to be as high as 1 in 90 in white populations. Onset normally occurs between 8 and 15 years of age, presenting as ataxia of gait accompanied by dysarthria...

OBJECTIVE To evaluate whether B-cell depletion before enzyme replacement therapy (ERT) initiation can block acid alpha-glucosidase (GAA) antibody responses and improve clinical outcomes. STUDY DESIGN Six subjects with Pompe disease (including 4 cross-reacting immunologic material-negative infants) aged 2-8 months received rituximab and sirolimus or mycophenolate before ERT. Four subjects cont...

Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disorder usually characterized by progressive early-onset ataxia. The most common ophthalmic manifestation of FRDA is optic neuropathy, which is usually late in onset, is slowly progressive, and rarely causes severe visual loss. The genetic basis of FRDA in most patients is the homozygous expansion of a GAA trinucleotide repea...

friedreich’s ataxia (fa) is the commonest genetic cause of ataxia and is associated with the expansion of a gaa repeat in intron 1 of the frataxin gene. iron accumulation in the mitochondria of patients with fa results in hypersensitivity to oxidative stress. mitochondrial dna (mtdna) could be considered a candidate modifier factor for fa disease since mitochondrial oxidative stress is thought ...

Methionine is an essential amino acid which when not incorporated into protein, can be converted to S-adenosylmethionine, the universal methyl donor in over 200 transmethylation reactions, which include creatine and phosphatidylcholine (PC) synthesis, as well as deoxyribonucleic acid (DNA) methylation. Following transmethylation, homocysteine is formed, which can be converted to cysteine via tr...

BACKGROUND Conventional and tissue Doppler echocardiographically derived myocardial velocity gradients (MVGs) were used to characterize the myocardium in patients with Friedreich's ataxia (FRDA), and the relationship between MVGs and the mutation in the FRDA gene, a GAA triplet repeat expansion, was investigated. METHODS AND RESULTS We studied 29 patients with FRDA (10 men, mean age 31+/-9 ye...

Introduction: The mechanisms involved in the virulence and recurrences of ocular toxoplasmosis are poorly understood. New detection methods are necessary to diagnose atypical cases and to better understand recurrences. A sensitive method to identify Toxoplasma gondii in peripheral blood of patients is necessary. Purpose: To detect the presence of T. gondii in peripheral human blood using immuno...

Enzyme replacement therapy (ERT) with recombinant human acid-α-glucosidase (rhGAA) is the only FDA approved therapy for Pompe disease. Without ERT, severely affected individuals (early onset) succumb to the disease within 2 years of life. A spectrum of disease severity and progression exists depending upon the type of mutation in the GAA gene (GAA), which in turn determines the amount of defect...