Gln-Val-Thr-Ser-Thr-Glu-Val |
Globose basal cells |
GDWIDSILAFSRSLHSLLVD |
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[globoid cell]
This term is used to refer to highly active, multinucleated phagocytes found in the brains of patients with Krabbe disease (globoid cell leukodystrophy) and in animal models of this disease (Suzuki, 1985; for a recent overview see also: Nicaise et al, 2016). This disease is an autosomal recessive brain white matter disease, a lysosomal storage disease caused by the deficiency of galactocerebrosidase (galactocerebroside beta-galactosidase; galactosylceramidase; EC3.2.1.46), which results in the accumulation in the brain of the neurotoxic sphingolipid intermediate psychosine (D-galactosyl-beta-1,1′-sphingosine or galactosylsphingosine) (Suzuki and Suzuki, 1970).
Globoid cells are found earlier than demyelination in globoid cell leukodystrophy and even before damage-associated inflammation
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