Distribution of cytoplasmic vacuoles in blood T and B lymphocytes in two lysosomal disorders.
Distribution of cytoplasmic vacuoles in purified T and B lymphocytes was analyzed in four cases of aspartylglucosaminuria (AGU) and in one case of neuronal ceroid lipofuscinosis (Spielmeyer-Sjörgren type). In all cases T cells were significantly more vacuolized than B cells. The ultrastructure of the cytoplasmic vacuoles was consistent with the concept of storage lysosomes. The cytoplasmic vacuoles both in T and B cells similar to abnormal lysosomes seen in the visceral organs in these diseases.
D000293:Adolescent; D000328:Adult; D054880:Aspartylglucosaminuria; D001402:B-Lymphocytes / Q000473:pathology; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology*; D008607:Intellectual Disability / Q000473:pathology; D008052:Lipid Metabolism, Inborn Errors / Q000473:pathology; D008062:Lipofuscin; D008214:Lymphocytes / Q000473:pathology*; D008247:Lysosomes; D008297:Male; D008661:Metabolism, Inborn Errors / Q000473:pathology*; D013601:T-Lymphocytes / Q000473:pathology; D014617:Vacuoles / Q000473:pathology*
D008062:Lipofuscin