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TRAPPC9 Analysis

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Mutation in Trafficking Protein Particle Complex 9 (TRAPPC9) is linked to moderate to severe forms of NonSyndromic Autosomal Recessive Mental Retardation (NS-ARMRA), a disease which affects mostly patients of Middle-Eastern descent. Patients afflicted by TRAPPC9 mutations exhibit secondary phenotypes associated with musculoskeletal defects. TRAPPC9 is a protein subunit of Transport Protein Particle II318, which is localized at the ER exit sites.424 TRAPPC9 is required for protein trafficking that recycle through the early endosome.51 TRAPPC9 is also known to play a role during the transport of endoplasmic (ER)-derived COPII vesicles toward the ER-Golgi intermediate compartment (ERGIC) through direct binding with p150Glued which plays an important …show more content…

183, 237, 249, 252 TRAPPC9 knockdown study using siRNA showed deficiency in nerve growth factor-induced neuronal differentiation of PC12 cells through the attenuation of the TNFα-induced NF-κB activation and decreases the expression Bcl-xL gene.167 These studies underline the involvement of TRAPPC9 in NF-κB pathway activation during nervous system …show more content…

NF-κB activation is known to be controlled through the canonical or classical 187 and non-canonical or alternate pathway333. The canonical activation is mediated through the translocation of the 65-kDa RelA and the 50-kDa NF-κB1 DNA-binding subunit, whereas, the non-canonical is thought to be mediated through the processing and translocation of the RelB complex. Emerging evidence shows that NF-κB activation negatively regulates osteoblast differentiation and function.7, 59 Inflammatory cytokines, in particular TNFα, provided the first indication of the inhibitor effect of the classical NF-κB pathway on bone formation in vivo and in vitro.129 The inhibitory effect of TNFα on osteoblast differentiation was mediated through Runx2 inhibition and was NF-kB dependent220. TNFα- and TNFR1- deficient mice showed increased basal bone mass in vivo and increased OB differentiation in vitro.220 Neil et al.7 showed that inhibition of NF-κB using S1627 NF-κB inhibitor increased osteoblast differentiation and bone formation through the up-regulation of osteoblast specific genes in vivo followed by an increased bone formation and repair bone defect in mouse calvarias critical defect model. The role on the non-classical NF-κB pathway in osteoblast was investigated by Chang et al.167 who showed that time and stage –specific inhibition of the IKK/NF-κB significantly increased

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