Pakistan Science Abstracts
Article details & metrics
No Detail Found!!
The Pathogenesis of Radial Ray Deficiency in Thrombocytopenia-absent Radius (TAR) Syndrome.
Author(s):
1. Mohammad Manna AlQattan: Department of Surgery, King Saud University, Riyadh, Saudi Arabia
Abstract:
The genetic basis of thrombocytopenia-absent radius (TAR) syndrome was recently identified to be related to the RBM8A gene. The encoded protein (known as the Y14 protein) is widely expressed in human cells (including osteoblasts) and plays several essential intracellular functions. Hence, the pathogenesis of radial ray deficiency in thrombocytopeniaabsent radius syndrome remains a mystery. The current paper reviews the pathogenesis of the clinical features of thrombocytopenia-absent radius syndrome and offers a hypothesis of pathogenesis through attenuation of the Fibroblast Growth Factor 8 signal in the mesoderm because of an increased degradation of the Fibroblast Growth Factor Receptor 1.
Page(s): 912-916
DOI: DOI not available
Published: Journal: Journal of College of Physicians and Surgeons--Pakistan : JCPSP, Volume: 26, Issue: 11, Year: 2016
Keywords:
Keywords are not available for this article.
References:
[1] GoldfarbCA,WustrackR,PrattJA,MenderA,ManskePR, 2007.Thumb function and appearance in thrombocytopenia absent radius syndrome,J Hand Surg Am 32 157 -61
[2] AlbersCA,Newbury-EcobR,OuwehandWH,GhevaertC, 2013.New insights into the genetic basis of TAR (thrombocytopeniaabsent radii) syndrome,Curr opinion Genet Develop 23 316 -23
[3] PapoulidisI,OikonomidouE,OrruS., 2014.Prenatal detection of TAR syndrome in a fetus with compound inheritance of an RBM8A SNP and a 334 kb deletion: A case report,Mol Med Rep 9 163 -5
[4] BottilloI,CastoriM,De Bernardo C., 2013.Prenatal diagnosis and post-mortem examination in a fetus with thrombocytopeniaabsent radius (TAR) syndrome due to compound heterozygosity for a 1q21.1 microdeletion and a RBM8Ahypomorphic allele: A case report,BMC Res Notes 22 376 -
[5] AlbersCA,PaulDS,SchulzeH, 2012.Compound inheritance of lowfrequency regulatory SNP and a rare null mutation in exonjunction complex subunit RBM8A causes TAR syndrome,Nat Genet 44 435 -9
[6] NicchiaE,GiordanoP,GrecoC,De RoccoD,SavoiaA, 2016.Molecular diagnosis of thrombocytopenia-absent radius syndrome using next-generation sequencing,Int J Lab Hematol 38 412 -8
[7] TassanoE,GimelliS,DiviziaMT, 2015.Thrombocytopenia-absent radius (TAR) syndrome due to compound inheritance for a 1q21.1 microdeletion and a low-frequency noncoding RBM8A SNP: A new familial case,Mol Cytogenet 5 8 -87
[8] YassaeeVR,Hashemi-GorjiF,SoltaniZ,Poorhosseini SM, 2014.A new approach for molecular diagnosis of TAR syndrome,Clin Biochem 47 835 -9
[9] SalicioniAM,XiM,VanderveerLA, 2000.Identifications and structural analysis of human RBM8A and RBM8B: Two highly conserved RNA-binding motif proteins that interact with OVCA1, a candidate tumour suppressor,Genomics 69 54 -62
[10] IshigakiY,NakamuraY,TatsunoT., 2013.Depletion of RNA-binding protein RBM8A (Y14) causes cell cycle deficiency and apoptosis in human cells,Exp Biol Med (Maywood) 238 889 -97
[11] ChuangTW,LeeKM,TarnWY, 2015.Function and pathological implications of exon junction complex factor Y14,Biomolecules 5 343 -55
[12] Le HirH,GatfieldD,IzaurraldeE,Moore MJ, 2001.nonsense-mediated mRNA decay,EMBO J 20 4987 -97
[13] Al-QattanMM,YangY,KozinSH, 2009.Embryology of the upper limb,J Hand Surg Am 34 1340 -50
[14] Lopez-RiosJ, 2016.The many lives of SHH in limb development and evolution,Semin Cell Dev Biol 49 116 -24
[15] Al-QattanMM, 2011.WNT pathways and upper limb anomalies,J Hand Surg Eur 36 9 -22
[16] WilliamsonI,LetticeLA,HillRE,BickmoreWA, 2016.Shh and ZRS enhancer colocalisation is specific to the zone of polarising activity,Development 143 2994 -3001
[17] Al-QattanMM,Al-SahabiA,Al-Arfaj N, 2012.Ulnar ray deficiency: A review of the classification systems, the clinical features in 72 cases and related developmental biology,J Hand Surg Eur 35 699 -707
[18] ObergKC,FeenstraJM,ManskePR,TonkinMA, 2010.Developmental biology and classification of congenital anomalies of the hand and upper extremity,J Hand Surg Am 35 2066 -76
[19] Al-QattanMM,Abou Al-Shaar H, 2015.Molecular basis of the clinical features of Holt-Oram syndrome resulting from missense and extended protein mutations of the TBX5 gene as well as TBX5 intragenic duplications,Gene 560 129 -36
[20] Al-QattanMM, 2013.Fancani anemia with concurrent thumb polydactyly and dorsal dimelia. A case report with discussion of embryology,Ann Plast Surg 70 116 -8
[21] BerginkS,JentschS., 2009.Principle of ubiquitin and SUMO modifications in DNA repair,Nature 458 461 -7
[22] Al-QattanMM, 2013.The ubiquitin/SUMO pathway and radial ray deficiency syndromes,Ann Plast Surg 70 240 -5
[23] BerginkS,JentschS., 2009.Principle of ubiquitin and SUMO modifications in DNA repair,Nature 458 461 -7
[24] GrunwaldM,BonoF, 2011.Structure of import in 13-ubc9 complex: Nuclear import and release of key regulator of sumoylation,EMBO J 30 427 -38
[25] IshigakiY,NakamuraY,TatsunoT., 2014.RNA-binding protein RBM8A (Y14) and MAGOH localize to centrosome in human A549 cells,Histochem Cell Biol 141 101 -9
[26] LiC,XuX,NelsonDK, 2005.FGFR1 function at the earliest stages of mouse limb development plays an indispensable role in subsequent autopod morphogenesis,Development 132 4755 -64
[27] NowakM,MachateA,YuSR,GuptaM,BrandM., 2011.Interpretation of the FGF8 morphogen gradient is regulated by endocytic trafficking,Nat Cell Biol 13 153 -8
[28] HaugstenEM,MaleckiJ,BjorklundSM,OlsnesS,WescheJ, 2008.Ubiquitination of fibroblast growth factor receptor-1 is required for its intracellular sorting but not for its endocytosis,Mol Biol Cell 19 3390 -403
[29] YuK,OrnitzDM, 2008.FGF signaling regulates mesenchymal differentiation and skeletal patterning along the limb bud proximodistal axis,Development 135 483 -91
[30] WilliamsDA,XuH,CancelasJA, 2006.Children are not little adults: Just ask their hematopoietic stem cells,J Clin Invest 116 2593 -6
[31] ZhaoM,RossJT,ItkinT., 2012.FGF signaling facilitates post-injury recovery of mouse hematopoietic system,Blood 120 1831 -42
[32] ParkHK,ParkHW,JeonSG, 2008.Distinct association of genetic variations of vascular endothelial growth factor, transforming growth factor-beta, and fibroblast growth factor receptors with atopy and airway hyper-responsiveness,Allergy 63 447 -53
[33] Dell-EraP,RoncaR,CocoL,NicoliS,MetraM,PrestaM., 2003.Fibroblast growth factor receptor 1 is essential for in vitro cardiomyocyte development,Cir Res 93 414 -20
[34] RoncaR,GualandiL,CresciniE,CalzaS,PrestaM,Dell-EraP, 2009.Fibroblast growth factor receptor-1 phosphorylation requirement for cardiomycyte differentiation in murine embryonic stem cells,J Cell Mol Med 13 1489 -98
[35] Di GiovanniV,WalkerKA,BushnellD., 2015.Fibroblast growth factor receptor-Frs 2α signaling is critical for nephron progenitors,Dev Biol 400 82 -93
[36] NguyenLS,KimHG,RosenfeldJA, 2013.Contribution of copy number variants involving nonsense-mediated mRNA decay pathway genes to neuro-developmental disorders,Hum Mol Genet 22 1816 -25
[37] KirovG,PocklingtonAJ,HolmansP, 2012.De novo CNV analysis implicates specific abnormalities of postsynaptic signalling complexes in the pathogenesis of schizophrenia,Mol Psychiatry 17 142 -53
[38] RosenfeldJA,TraylorRN,SchaferGB, 2012.Proximal microdeletions and microduplications of 1q21.1 contribute to variable abnormal phenotypes,Eur J Hum Genet 20 754 -61
[39] BaronO,ForthmannB,LeeYW, 2012.Cooperation of nuclear fibroblast growth factor receptor 1 and Nurr 1 offers new interactive mechanism in postmitotic development of mesencephalic dopaminergic neurons,J Biol Chem 287 19827 -40
[40] SunX,MarianiFV,MartinGR, 2002.Functions of FGF signaling from the apical ectodermal ridge in limb development,Nature 418 501 -8
[41] GreenhalghKL,HowellRT,BottaniA, 2002.Thrombocytopenia - absent radius syndrome: A clinical genetic study,J Med Genet 39 876 -81
Citations
Citations are not available for this document.
0

Citations

0

Downloads

6

Views