Educator Readiness Evaluation in the Implementation of Curriculum 2013 in Disadvantaged, Frontier, and Outermost Areas

This study aims to describe the readiness of educators in implementing the 2013 curriculum in the 3T area in North Gorontalo District. The method used in this research is descriptive qualitative method. The subjects in this study were school principals and educators in the 3T area school. The research result shows four things; first; Academic qualifications of educators in schools in the 3Tl area have not been fully fulfilled according to Law No. 20 of 2003 concerning the National Education System Article 42 paragraph (1). Second; The Academic Competence of Educators in the 3T area is not yet fully educated with S1 academic qualifications in accordance with Article 8 of Law Number 14 of 2005. The three certificates of educators have not been fully fulfilled in accordance with the mandate of Law Number 14 of 2005 concerning Educators and Lecturers. The fourth role of educators in realizing the goals of national education principals continues to encourage educators to continue to carry out learning innovations, especially in the implementation of the 2013 curriculum as an effort to improve professionalism as a form of role in realizing the goals of national education.

Molecular Characterization of Rare Genodermatoses in Pakistani Families.

Genodermatoses include a large group of inherited dermatological disorders that is often present with multisystem involvement resulting in various clinical manifestations due to genetic heterogeneity with pathogenic variations in more than 500 genes. Most genodermatoses occur early, during the neonatal period, infancy or early childhood, some conditions may appear later in life during adolescence or adulthood. Diagnosis of genodermatoses has been a major challenge and requires both clinical and investigational correlation to reach a diagnosis. Sometimes, even with comprehensive investigations, a definitive diagnosis cannot be achieved. With the arrival of new investigational. methods such as next generation sequencing, which not only successfully identified the molecular basis of many genodermatoses but also play important role in clinical use to help in the diagnosis of patients with suspected genodermatoses. In the current study, whole exome sequencing and Sanger sequencing were used to investigate genetic defects of nine Pakistani families affected with pseudoxanthoma elasticum (Families: A and B) and epidermolysis bullosa (Families: C,D E,F,G,H,I). Whole exome sequencing identified a novel homozygous frameshift variant (c.1799_1805dupGTCTGGT; p. Thr603fs*11) in ABCC6 in family A, a compound heterozygous variant (c.2294G>A; p.Arg765Gln and c.2974G>A; p.Gly992Arg) in ABCC6 in family B, a single nucleotide insertion variant (c.151insG; p.Gln226fs*) in LAMA3 in family C, two novel missense variants c.1285G>T (p.Asp429Tyr) and c.3373G>A (p.Gly1125Ser) in ITGB4 in family D, a novel homozygous missense variant c.1828A>G (p.Arg610Gly) in PLEC in family E, a heterozygous missense variant c.6209G>A (p.Gly2070Glu) in COL7A1 in family F, a homozygous frameshift variant c.676dupC (p.Gln226fs) in FERMT1 in family G, a homozygous nonsense variant c. C1705T in LAMB3 in family H and a homozygous nonsense variant c.C1573T (p.R525X) in COL7A1 in family I. In conclusion, two families affected with pseudoxanthoma elasticum and seven families affected with epidermolysis bullosa were identified with novel variants in ABCC6, ITGB4, and PLEC1. It should be assistive in the genetic counseling and prenatal diagnosis of the families as well as for the designing of improved diagnostic and therapeutic approaches.