Degeneration of neural cells in the central nervous system of mice deficient in the gene for the adhesion molecule on Glia, the beta 2 subunit of murine Na,K-ATPase

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Degeneration of neural cells in the central nervous system of mice deficient in the gene for the adhesion molecule on Glia, the beta 2 subunit of murine Na,K-ATPase. / Magyar, J P; Bartsch, U; Wang, Z Q; Howells, N; Aguzzi, A; Wagner, E F; Schachner, M.

In: J CELL BIOL, Vol. 127, No. 3, 11.1994, p. 835-45.

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@article{627fa14bc08d4d6d96239a791326b7d8,
title = "Degeneration of neural cells in the central nervous system of mice deficient in the gene for the adhesion molecule on Glia, the beta 2 subunit of murine Na,K-ATPase",
abstract = "We generated mice, null mutant in the adhesion molecule on glia (AMOG), the beta 2 subunit of the murine Na,K-ATPase gene. These mice exhibit motor incoordination at 15 d of age, subsequently tremor and paralysis of extremities, and die at 17-18 d after birth. At these ages, the mutants have enlarged ventricles, degenerating photoreceptor cells, and swelling and degeneration of astrocytic endfeet, leading to vacuoles adjoining capillaries of brain stem, thalamus, striatum, and spinal cord. In tissue homogenates from entire brains of 16-17-d-old mutants, Na,K-ATPase activity and expression of the beta 1 subunit of the Na,K-ATPase and of the neural adhesion molecules L1, N-CAM, and MAG appear normal. We suggest that the mutant phenotype can be related primarily to reduced pump activity, with neural degeneration as a possible consequence of osmotic imbalance.",
keywords = "Adenosine Triphosphatases, Amino Acid Sequence, Animals, Base Sequence, Blotting, Southern, Brain, Capillaries, Cation Transport Proteins, Cell Adhesion Molecules, Neuronal, Cerebellar Cortex, Cerebrovascular Circulation, Cloning, Molecular, Embryo, Mammalian, Extracellular Matrix Proteins, In Situ Hybridization, Macromolecular Substances, Mice, Mice, Neurologic Mutants, Microscopy, Electron, Molecular Sequence Data, Motor Activity, Nerve Degeneration, Neuroglia, Neurons, Oligodeoxyribonucleotides, Polymerase Chain Reaction, Restriction Mapping, Sodium-Potassium-Exchanging ATPase, Spinal Cord, Stem Cells, Vacuoles, Journal Article, Research Support, Non-U.S. Gov't",
author = "Magyar, {J P} and U Bartsch and Wang, {Z Q} and N Howells and A Aguzzi and Wagner, {E F} and M Schachner",
year = "1994",
month = nov,
language = "English",
volume = "127",
pages = "835--45",
journal = "J CELL BIOL",
issn = "0021-9525",
publisher = "Rockefeller University Press",
number = "3",

}

RIS

TY - JOUR

T1 - Degeneration of neural cells in the central nervous system of mice deficient in the gene for the adhesion molecule on Glia, the beta 2 subunit of murine Na,K-ATPase

AU - Magyar, J P

AU - Bartsch, U

AU - Wang, Z Q

AU - Howells, N

AU - Aguzzi, A

AU - Wagner, E F

AU - Schachner, M

PY - 1994/11

Y1 - 1994/11

N2 - We generated mice, null mutant in the adhesion molecule on glia (AMOG), the beta 2 subunit of the murine Na,K-ATPase gene. These mice exhibit motor incoordination at 15 d of age, subsequently tremor and paralysis of extremities, and die at 17-18 d after birth. At these ages, the mutants have enlarged ventricles, degenerating photoreceptor cells, and swelling and degeneration of astrocytic endfeet, leading to vacuoles adjoining capillaries of brain stem, thalamus, striatum, and spinal cord. In tissue homogenates from entire brains of 16-17-d-old mutants, Na,K-ATPase activity and expression of the beta 1 subunit of the Na,K-ATPase and of the neural adhesion molecules L1, N-CAM, and MAG appear normal. We suggest that the mutant phenotype can be related primarily to reduced pump activity, with neural degeneration as a possible consequence of osmotic imbalance.

AB - We generated mice, null mutant in the adhesion molecule on glia (AMOG), the beta 2 subunit of the murine Na,K-ATPase gene. These mice exhibit motor incoordination at 15 d of age, subsequently tremor and paralysis of extremities, and die at 17-18 d after birth. At these ages, the mutants have enlarged ventricles, degenerating photoreceptor cells, and swelling and degeneration of astrocytic endfeet, leading to vacuoles adjoining capillaries of brain stem, thalamus, striatum, and spinal cord. In tissue homogenates from entire brains of 16-17-d-old mutants, Na,K-ATPase activity and expression of the beta 1 subunit of the Na,K-ATPase and of the neural adhesion molecules L1, N-CAM, and MAG appear normal. We suggest that the mutant phenotype can be related primarily to reduced pump activity, with neural degeneration as a possible consequence of osmotic imbalance.

KW - Adenosine Triphosphatases

KW - Amino Acid Sequence

KW - Animals

KW - Base Sequence

KW - Blotting, Southern

KW - Brain

KW - Capillaries

KW - Cation Transport Proteins

KW - Cell Adhesion Molecules, Neuronal

KW - Cerebellar Cortex

KW - Cerebrovascular Circulation

KW - Cloning, Molecular

KW - Embryo, Mammalian

KW - Extracellular Matrix Proteins

KW - In Situ Hybridization

KW - Macromolecular Substances

KW - Mice

KW - Mice, Neurologic Mutants

KW - Microscopy, Electron

KW - Molecular Sequence Data

KW - Motor Activity

KW - Nerve Degeneration

KW - Neuroglia

KW - Neurons

KW - Oligodeoxyribonucleotides

KW - Polymerase Chain Reaction

KW - Restriction Mapping

KW - Sodium-Potassium-Exchanging ATPase

KW - Spinal Cord

KW - Stem Cells

KW - Vacuoles

KW - Journal Article

KW - Research Support, Non-U.S. Gov't

M3 - SCORING: Journal article

C2 - 7525597

VL - 127

SP - 835

EP - 845

JO - J CELL BIOL

JF - J CELL BIOL

SN - 0021-9525

IS - 3

ER -