TY - JOUR
T1 - Neurotransmitter chemistry in feline GM1 gangliosidosis
T2 - A model for human ganglioside storage disease
AU - Singer, Harvey S.
AU - Coyle, Joseph T.
AU - Weaver, Debbie L.
AU - Kawamura, Nariko
AU - Baker, Henry J.
PY - 1982/7
Y1 - 1982/7
N2 - Assays for synaptosomal high‐affinity uptake activity (glutamate, γ‐aminobutyric acid, norepinephrine), neurotransmitter synthesizing enzymes (choline acetyltransferase, glutamate decarboxylase, tyrosine hydroxylase), and endogenous neurotransmitters were performed in cats with advanced inherited GM1 gangliosidosis. A significant reduction in uptake activity, ranging from 24 to 77% of control, was demonstrated in motor, occipital, and cerebellar brain regions. This reduction was unassociated with comparable alterations in neurotransmitter levels or synthesizing enzyme activity. We hypothesize that the defect of neurotransmitter inactivation is part of an overall abnormality of synaptic membrane function that could contribute to the neurological symptoms seen in the hereditary gangliosidoses.
AB - Assays for synaptosomal high‐affinity uptake activity (glutamate, γ‐aminobutyric acid, norepinephrine), neurotransmitter synthesizing enzymes (choline acetyltransferase, glutamate decarboxylase, tyrosine hydroxylase), and endogenous neurotransmitters were performed in cats with advanced inherited GM1 gangliosidosis. A significant reduction in uptake activity, ranging from 24 to 77% of control, was demonstrated in motor, occipital, and cerebellar brain regions. This reduction was unassociated with comparable alterations in neurotransmitter levels or synthesizing enzyme activity. We hypothesize that the defect of neurotransmitter inactivation is part of an overall abnormality of synaptic membrane function that could contribute to the neurological symptoms seen in the hereditary gangliosidoses.
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U2 - 10.1002/ana.410120107
DO - 10.1002/ana.410120107
M3 - Article
C2 - 6126152
AN - SCOPUS:0019945077
SN - 0364-5134
VL - 12
SP - 37
EP - 41
JO - Annals of neurology
JF - Annals of neurology
IS - 1
ER -