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There are many reports regarding to effects of Granulocyte colony-stimulating factor [G-CSF] and stem cell factor [SCF]alone in liver repair .But conflicting data have been reported regarding the role of growth factors such as G-CSF and SCF in the liver regeneration system. Also, there is not such data regarding to effects of co-administration both of G-CSF and SCF in the liver damage condition. An experimental model of rat liver damage induced by the thioacetamide. Five different groups of animals receiving 0.9% NaCl, TAA alone, TAA + G-CSF, TAA + SCF and TAA + [G-CSF+SCF].The activity of glutamate pyruvate transaminase [GPT/AlT]and glutamate oxaloacetate transaminase [GOT/AST] were measured after the thioacetamide [TAA] injection and the administration of combination of G-CSF +SCF for 12 weeks. Also histological tests were carried out at the end experiments. The pre-treatment of combination of G-CSF and SCF for 12 weeks reduced the degree of liver injury. The mean of GOT activity was 61.24 [U/L] in the G-CSF +SCF and versus 132.86 in the TAA-alone group. These differences in the GOT activity were statistically significant [P<0.05]. Also, in the G-CSF +SCF and TAA group the mean of GPT activity [4.35 versus 11.79, respectively] were lower than in the TAA-alone group, this difference was statistically significant [P<0.05]. Liver sections from a rat treated only with TAA, showing damage, but TAA and G-CSF + SCF no significant damage is present. On the other hand histological results revealed a very mild degree of inflammation were observed in the livers of the combination of G-SCF+SCF and TAA-treated rats compared to TAA only treated group. Biochemical and microscopic analysis revealed that combination of G-CSF and SCF pre-treatment significantly enhances liver regeneration after TAA-induced liver injury
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<p><b>INTRODUCTION</b>Childhood-onset proximal spinal muscular atrophies (SMAs) are an autosomal recessive, clinically heterogeneous group of neuropathies characterised by the selective degeneration of anterior horn cells. SMA has an estimated incidence of 1 in 10,000 live births. The causative genes are survival motor neuron (SMN) gene and neuronal apoptosis inhibitory protein (NAIP) gene. Deletions of the telomeric copy of SMN gene (SMN1) have been reported in 88.5% to 95% of SMA cases, whereas the deletion rate for NAIP gene (NAIP) is between 20% and 50% depending on the disease severity. The main objective of this study was to genetically characterise the childhood onset of SMA in Iran.</p><p><b>MATERIALS AND METHODS</b>Molecular analysis was performed on a total of 75 patients with a clinical diagnosis of SMA. In addition to common PCR analysis for SMN1 exons 7 and 8, we analysed NAIP exons 4 and 5, along with exon 13, as a internal control, by bi-plex PCR.</p><p><b>RESULTS</b>The homozygous-deletion frequency rate for the telomeric copy of SMN exons 7 and 8 in all types of SMA was 97%. Moreover, exons 5 and 6 of NAIP gene were deleted in approximately 83% of all SMA types. Three deletion haplotypes were constructed by using SMN and NAIP genotypes. Haplotype A, in which both genes are deleted, was seen in approximately 83% of SMA types I and II but not type III. It was also found predominantly in phenotypically severe group with an early age of onset (i.e., less than 6-month-old). We also report 34 of our prenatal diagnosis.</p><p><b>CONCLUSIONS</b>To our knowledge, the present study is the first one giving detailed information on SMN and NAIP deletion rates in Iranian SMA patients. Our results show that the frequency of SMN1 homozygous deletions in Iran is in agreement with previous studies in other countries. The molecular analysis of SMA-related gene deletion/s will be a useful tool for pre- and postnatal diagnostic.</p>