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provides coniferyl ferulate(CAS#:2142-01-0) MSDS, density, melting point, boiling point, structure, formula, molecular weight etc. Articles of coniferyl ferulate are included as well.>> amp version: coniferyl ferulate
Brr2 is an RNA-dependent ATPase required to unwind the U4/U6 snRNA duplex during spliceosome assembly. Mutations within the ratchet helix of the Brr2 RNA binding channel result in a form of degenerative human blindness known as retinitis pigmentosa (RP). The biochemical consequences of these mutations on Brr2’s RNA binding, helicase, and ATPase activity have not yet been characterized. Therefore, we identified the largest construct of Brr2 that is soluble in vitro, which truncates the first 247 amino acids of the N terminus (Δ247-Brr2), to characterize the effects of the RP mutations on Brr2 activity. The Δ247-Brr2 RP mutants exhibit a gradient of severity of weakened RNA binding, reduced helicase activity, and reduced ATPase activity compared with wild type Δ247-Brr2. The globular C-terminal Jab1/Mpn1-like domain of Prp8 increases the ability of Δ247-Brr2 to bind the U4/U6 snRNA duplex at high pH and increases Δ247-Brr2’s RNA-dependent ATPase activity and the extent of RNA unwinding. However, this domain of Prp8 does not differentially affect the Δ247-Brr2 RP mutants compared with the wild type Δ247-Brr2. When stimulated by Prp8, wild type Δ247-Brr2 is able to unwind long stable duplexes in vitro, and even the RP mutants capable of binding RNA with tight affinity are incapable of fully unwinding short duplex RNAs. Our data suggest that the RP mutations within the ratchet helix impair Brr2 translocation through RNA helices.
ATPase, RNA helicase, RNA splicing, RNA-Protein interaction, spliceosome, U4/U6 snRNA, retinitis pigmentosa
Retinitis Pigmentosa Mutations in Bad Response to Refrigeration 2 (Brr2) Impair ATPase and Helicase Activity*♦
Sarah Ledoux and Christine Guthrie1
2016 Jun 3
There are three common C2 protein alleles in caucasians, C2*C, C2*B, and C2*Q0, with allele frequencies of 0.96, 0.03, and 0.01, as well as Sst I RFLP variants of 2.75, 2.7, 2.65, 2.55, and 2.4 kb, with frequencies of 0.017, 0.533, 0.358, 0.017, and 0.075. Thus, C2*C is informatively split by the RFLP. Of 94 nonrandomly ascertained caucasian complotypes, 77 contained C2*C, four contained C2*Q0, and 13 had C2*B. None of the C2*C-containing complotypes carried the 2.75 kb Sst I fragment and all of the complotypes with C2*B or C2*Q0 carried it. All of the C2*Q0 alleles were associated with C4A*4, C4B*2 in the complotype S042 as previously reported. C2*B was usually (9/13) in the complotype SB42, occasionally (1/13 each) in SB45, SB41, SB(4,3)0, and SB31. Thus, the association of the C2 2.75-kb fragment was with C2*B and C2*Q0, not with C4A*4, C4B*2, or even C4A*4 alone. The complotype SC42 was associated with the 2.65-kb Sst I fragment in four of five instances and in a single example with the 2.7-kb fragment. C2*B and C2*Q0 possibly had a common evolutionary ancestor complotype which carried the 2.75-kb Sst I fragment, and BF*S, C4A*4, and C4B*2. C2*B (particularly as the haplotype HLA-Bw62, SB42, DR4) is associated with type 1 diabetes but C2*Q0 is protective.
A restriction fragment of the C2 gene is a unique marker for C2 deficiency and the uncommon C2 allele C2*B (a marker for type 1 diabetes).
S Simon, Z Awdeh, R D Campbell, P Ronco, 2nd, S J Brink, G S Eisenbarth, E J Yunis, and C A Alper
Progress in health has occurred in the past decades in Cambodia, in terms of health service access and interventions, but several indicators, including the prevalence of malnourished children, remain alarming. The causes of undernutrition are often linked to inadequate access to water, sanitation and hygiene services but limited evidence exists on the direct association between poor WASH practices and children’s’ nutritional statuses. This study investigates the relationship between water, sanitation and hygiene practices, defined as the child-sensitive composite score, and the nutritional status of children under five years old, measured as the weight-for-height z-score, mid-upper arm circumference or height-for-age z-score in six districts of Cambodia. The analysis used data from a longitudinal study, comprising extensive data collection on anthropometry, health, nutrition, WASH, and cognitive development. Chronological trends in wasting and stunting were described cross-sectionally, whereas the effect of WASH practices on the nutritional status of children over up to three consecutive study visits was examined with a linear mixed-effects model. The prevalence of wasting decreased during the study while stunting prevalence increased. A small, but significant, association was found between the WASH child-sensitive composite scores and the wasting child anthropometry indicators: weight-for-height z-score or mid-upper arm circumference. Evidence for an association with height-for-age z-score, detecting stunted children, was found when the independent variable was quantified according to global, but not national, guidelines. This study reinforces discordant existing evidence towards a direct association between WASH practices and children’s nutritional status, suggesting the need to align nutrition and WASH programmes.
weight-for-height z-score, mid-upper arm circumference, height-for-age z-score, WASH, child-sensitive, WASH composite score
Child-Sensitive WASH Composite Score and the Nutritional Status in Cambodian Children
Giulia Manzoni,1,* Arnaud Laillou,2 Chea Samnang,3 Rathmony Hong,2 Frank T. Wieringa,4 Jacques Berger,4 Etienne Poirot,2 and Francesco Checchi1