Biomaterial Database

Developmental expression of FMRP in the astrocyte lineage: implications for fragile X syndrome. 2007

Laura K K Pacey, and Laurie C Doering

Department of Pathology and Molecular Medicine, Health Science Centre, HSC 1R1, McMaster University, 1200 Main Street West, Hamilton, Ontario, Canada L8N 3Z5.

One of the most common causes of mental retardation in humans, Fragile X syndrome, results from the absence of FMRP, the protein product of the FMR1 gene. In the nervous system, expression of FMRP has been thought to be confined mainly to neurons as little research has examined FMRP expression in non-neuronal lineages. We present evidence that, in addition to neuronal expression, FMRP is expressed in developing CNS glial cells in vitro and in vivo. The neurosphere assay was used to establish cultures of stem and progenitor cells from the brains of wildtype and FMRP knockout (B6.129.FMR1/FvBn) mouse pups. When the neurospheres were differentiated in vitro, approximately 50% of the FMRP positive cells also expressed GFAP. Immunocytochemical studies of the embryonic and postnatal mouse brain revealed coexpression of FMRP and GFAP in the developing hippocampus. Prominent coexpression was also observed in ependymal cells surrounding the third ventricle and astrocytes of the glia limitans. No double-labeled cells were evident in the brains of young adult mice. Cells coexpressing FMRP and the oligodendrocyte precursor marker NG2 were also identified in the hippocampus and corpus callosum of the early postnatal brain. Our results suggest that FMRP is expressed in cells of non-neuronal lineage(s) during development. This represents potential involvement of glial cells in the neural development of fragile X syndrome.

UI	MeSH Term	Description	Entries
D009474	Neurons	The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM.	Nerve Cells,Cell, Nerve,Cells, Nerve,Nerve Cell,Neuron
D009836	Oligodendroglia	A class of large neuroglial (macroglial) cells in the central nervous system. Oligodendroglia may be called interfascicular, perivascular, or perineuronal (not the same as SATELLITE CELLS, PERINEURONAL of GANGLIA) according to their location. They form the insulating MYELIN SHEATH of axons in the central nervous system.	Interfascicular Oligodendroglia,Oligodendrocytes,Perineuronal Oligodendroglia,Perineuronal Satellite Oligodendroglia Cells,Perivascular Oligodendroglia,Satellite Cells, Perineuronal, Oligodendroglia,Perineuronal Satellite Oligodendrocytes,Interfascicular Oligodendroglias,Oligodendrocyte,Oligodendrocyte, Perineuronal Satellite,Oligodendrocytes, Perineuronal Satellite,Oligodendroglia, Interfascicular,Oligodendroglia, Perineuronal,Oligodendroglia, Perivascular,Perineuronal Satellite Oligodendrocyte,Satellite Oligodendrocyte, Perineuronal,Satellite Oligodendrocytes, Perineuronal
D001921	Brain	The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.	Encephalon
D002454	Cell Differentiation	Progressive restriction of the developmental potential and increasing specialization of function that leads to the formation of specialized cells, tissues, and organs.	Differentiation, Cell,Cell Differentiations,Differentiations, Cell
D002473	Cell Wall	The outermost layer of a cell in most PLANTS; BACTERIA; FUNGI; and ALGAE. The cell wall is usually a rigid structure that lies external to the CELL MEMBRANE, and provides a protective barrier against physical or chemical agents.	Cell Walls,Wall, Cell,Walls, Cell
D005600	Fragile X Syndrome	A condition characterized genotypically by mutation of the distal end of the long arm of the X chromosome (at gene loci FRAXA or FRAXE) and phenotypically by cognitive impairment, hyperactivity, SEIZURES, language delay, and enlargement of the ears, head, and testes. INTELLECTUAL DISABILITY occurs in nearly all males and roughly 50% of females with the full mutation of FRAXA. (From Menkes, Textbook of Child Neurology, 5th ed, p226)	FRAXA Syndrome,FRAXE Syndrome,Martin-Bell Syndrome,Fra(X) Syndrome,Fragile X Mental Retardation Syndrome,Fragile X-F Mental Retardation Syndrome,Mar (X) Syndrome,Marker X Syndrome,Mental Retardation, X-Linked, Associated With Fragile Site Fraxe,Mental Retardation, X-Linked, Associated With Marxq28,X-Linked Mental Retardation and Macroorchidism,FRAXA Syndromes,FRAXE Syndromes,Fragile X Syndromes,Marker X Syndromes,Martin Bell Syndrome,Syndrome, FRAXA,Syndrome, FRAXE,Syndrome, Fragile X,Syndrome, Marker X,Syndrome, Martin-Bell,Syndromes, FRAXA,Syndromes, FRAXE,Syndromes, Fragile X,Syndromes, Marker X,X Linked Mental Retardation and Macroorchidism
D005819	Genetic Markers	A phenotypically recognizable genetic trait which can be used to identify a genetic locus, a linkage group, or a recombination event.	Chromosome Markers,DNA Markers,Markers, DNA,Markers, Genetic,Genetic Marker,Marker, Genetic,Chromosome Marker,DNA Marker,Marker, Chromosome,Marker, DNA,Markers, Chromosome
D005904	Glial Fibrillary Acidic Protein	An intermediate filament protein found only in glial cells or cells of glial origin. MW 51,000.	Glial Intermediate Filament Protein,Astroprotein,GFA-Protein,Glial Fibrillary Acid Protein,GFA Protein
D000818	Animals	Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA.	Animal,Metazoa,Animalia
D001253	Astrocytes	A class of large neuroglial (macroglial) cells in the central nervous system - the largest and most numerous neuroglial cells in the brain and spinal cord. Astrocytes (from "star" cells) are irregularly shaped with many long processes, including those with "end feet" which form the glial (limiting) membrane and directly and indirectly contribute to the BLOOD-BRAIN BARRIER. They regulate the extracellular ionic and chemical environment, and "reactive astrocytes" (along with MICROGLIA) respond to injury.	Astroglia,Astroglia Cells,Astroglial Cells,Astrocyte,Astroglia Cell,Astroglial Cell,Astroglias,Cell, Astroglia,Cell, Astroglial