“
“The authors would like to correct the omission of a reference in their discussion of the genetic interaction between Sdo1 and Tif6 in yeast. This work was published by Menne TF, Goyenechea B, Sanchez-Puig N, Wong, C. C., Tonkin, L. M., Ancliff, P. J., Brost, R. L., Costanzo, M., Boone, C., Warren, A. J.. The Shwachman–Bodian–Diamond GSK1120212 concentration syndrome protein mediates translational activation of ribosomes in yeast. Nat Genet. 2007;39:486–495. “
“The authors regret that the above article contained a minor error on page 2: the twenty first line of the right-hand column should read, “In normal red cells the ratio between reduced and oxidized glutathione (GSSG)
is usually about 100:1” as opposed to “In normal red cells the ratio between oxidized and reduced glutathione (GSSH) is 100:1”. “
“Sickle cell disease (SCD), the most common inherited red blood cell (RBC) disorder, affects individuals of African, Mediterranean, and Asian descent and manifests as haemolysis and vaso-occlusion [1]. Patients experience a
spectrum of disease symptoms and complications, including periods of acute pain (vaso-occlusive episodes [VOE]), chronic pain, multi-organ injury, reduced quality of life, and a shortened lifespan [1] and [2]. Worldwide it is estimated that over 200,000 children affected with SCD are born every year, primarily in selleck chemical sub-Saharan Africa (180,000 births per year) [3] and [4]. Approximately 2000 children in the US [5] are born with SCD each year, with a disease incidence of 1 in 2474 live births (newborn screening data 1990–1999) [6]; the estimated US prevalence ranges from 70,000–140,000 [7] and [8]. Among individuals with the homozygous sickle haemoglobin mutation (HbSS) living in first-world countries, the estimated mean life expectancy Tacrolimus (FK506) is 39 years [8], which has improved significantly over the last few decades. Increased overall survival of paediatric patients with SCD [9] (Fig. 1) can be attributed to the landmark Prophylactic Penicillin Study (PROPS; 1986), [10] which demonstrated that the use of prophylactic
penicillin could prevent life-threatening infections in affected children. Thus, universal newborn screening became standard practice in the US in the late 1990s and in the United Kingdom in the early 2000′s [10], [11] and [12], enabling early diagnosis and patient management. The introduction of a pneumococcal conjugate vaccine also significantly contributes to decreased SCD mortality in children younger than 10 years of age [12] and [13]. However, in low-resource countries, more than 50% of children younger than 5 years of age die due to complications of SCD [14]. Because more than 98% of children with milder forms of SCD in high-resource countries are living into adulthood, SCD is now a chronic condition requiring comprehensive, life-long management [9].