Biomaterial Database

Responses to cyclic AMP is impaired in the twitcher Schwann cells in vitro. 1999

H Yamada, and K Suzuki

Department of Pathology and Laboratory Medicine, and Neuroscience Center, School of Medicine, CB No. 7525, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599-7525, USA.

Twitcher (twi/twi) is a murine model of genetic demyelinating disease globoid cell leukodystrophy (GLD). Available data suggest that demyelination in GLD is caused by degeneration or dysfunction of myelin-forming cells resulting from an accumulation of psychosine, a toxic substrate of galactosylceramidase and a potent inhibitor of protein kinase C (PKC). We investigated proliferation and differentiation of twi/twi Schwann cells in response to forskolin, an adenylate cyclase activator. In twi/twi Schwann cells isolated at the postnatal day (P) 10 prior to the onset of demyelination, proliferation and an expression of the surface galactocerebroside (galC) in response to forskolin were similar to those of +/+ mice. However, in twi/twi Schwann cells isolated from demyelinating sciatic nerves at P20 or P30, fewer numbers of cells expressed surface galC compared to age matched control (+/+) Schwann cells. In all Schwann cells, surface galC expression was lost after 3 days in vitro (DIV). However, with an administration of 50 microM forskolin in the media containing 1% fetal bovine serum (FBS) on the 4 DIV, surface galC could be reexpressed in all +/+ and P10 twi/twi Schwann cells but not in P20 or P30 twi/twi cells. In the media containing 10% FBS, forskolin also stimulated proliferation of Schwann cells from P10 twi/twi, and P10 and P30+/+ mice but not those from P30 twi/twi mice. These results are consistent with a metabolic perturbation of twi/twi Schwann cells that may be reflecting cellular dysfunctions by inhibition of the PKC.

UI	MeSH Term	Description	Entries
D008810	Mice, Inbred C57BL	One of the first INBRED MOUSE STRAINS to be sequenced. This strain is commonly used as genetic background for transgenic mouse models. Refractory to many tumors, this strain is also preferred model for studying role of genetic variations in development of diseases.	Mice, C57BL,Mouse, C57BL,Mouse, Inbred C57BL,C57BL Mice,C57BL Mice, Inbred,C57BL Mouse,C57BL Mouse, Inbred,Inbred C57BL Mice,Inbred C57BL Mouse
D009186	Myelin Sheath	The lipid-rich sheath surrounding AXONS in both the CENTRAL NERVOUS SYSTEMS and PERIPHERAL NERVOUS SYSTEM. The myelin sheath is an electrical insulator and allows faster and more energetically efficient conduction of impulses. The sheath is formed by the cell membranes of glial cells (SCHWANN CELLS in the peripheral and OLIGODENDROGLIA in the central nervous system). Deterioration of the sheath in DEMYELINATING DISEASES is a serious clinical problem.	Myelin,Myelin Sheaths,Sheath, Myelin,Sheaths, Myelin
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
D002455	Cell Division	The fission of a CELL. It includes CYTOKINESIS, when the CYTOPLASM of a cell is divided, and CELL NUCLEUS DIVISION.	M Phase,Cell Division Phase,Cell Divisions,Division Phase, Cell,Division, Cell,Divisions, Cell,M Phases,Phase, Cell Division,Phase, M,Phases, M
D000242	Cyclic AMP	An adenine nucleotide containing one phosphate group which is esterified to both the 3'- and 5'-positions of the sugar moiety. It is a second messenger and a key intracellular regulator, functioning as a mediator of activity for a number of hormones, including epinephrine, glucagon, and ACTH.	Adenosine Cyclic 3',5'-Monophosphate,Adenosine Cyclic 3,5 Monophosphate,Adenosine Cyclic Monophosphate,Adenosine Cyclic-3',5'-Monophosphate,Cyclic AMP, (R)-Isomer,Cyclic AMP, Disodium Salt,Cyclic AMP, Monoammonium Salt,Cyclic AMP, Monopotassium Salt,Cyclic AMP, Monosodium Salt,Cyclic AMP, Sodium Salt,3',5'-Monophosphate, Adenosine Cyclic,AMP, Cyclic,Adenosine Cyclic 3',5' Monophosphate,Cyclic 3',5'-Monophosphate, Adenosine,Cyclic Monophosphate, Adenosine,Cyclic-3',5'-Monophosphate, Adenosine,Monophosphate, Adenosine Cyclic
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
D012583	Schwann Cells	Neuroglial cells of the peripheral nervous system which form the insulating myelin sheaths of peripheral axons.	Schwann Cell,Cell, Schwann,Cells, Schwann
D015290	Second Messenger Systems	Systems in which an intracellular signal is generated in response to an intercellular primary messenger such as a hormone or neurotransmitter. They are intermediate signals in cellular processes such as metabolism, secretion, contraction, phototransduction, and cell growth. Examples of second messenger systems are the adenyl cyclase-cyclic AMP system, the phosphatidylinositol diphosphate-inositol triphosphate system, and the cyclic GMP system.	Intracellular Second Messengers,Second Messengers,Intracellular Second Messenger,Messenger, Second,Messengers, Intracellular Second,Messengers, Second,Second Messenger,Second Messenger System,Second Messenger, Intracellular,Second Messengers, Intracellular,System, Second Messenger,Systems, Second Messenger
D051379	Mice	The common name for the genus Mus.	Mice, House,Mus,Mus musculus,Mice, Laboratory,Mouse,Mouse, House,Mouse, Laboratory,Mouse, Swiss,Mus domesticus,Mus musculus domesticus,Swiss Mice,House Mice,House Mouse,Laboratory Mice,Laboratory Mouse,Mice, Swiss,Swiss Mouse,domesticus, Mus musculus