PMID: 7085893May 1, 1982Paper

Distribution pattern of lysosomal granules in fibroblasts of the Chediak-Higashi syndrome

Journal of Clinical Pathology
K AbeM Honma

Abstract

Cultured fibroblasts from a patient with the Chediak-Higashi syndrome, the mother of the patient, and a normal control were studied by light and electron microscopy. The distribution pattern of PAS-positive and acid phosphatase-containing granules in the cytoplasm differed significantly in the fibroblasts from the patient when compared with those from the mother and control. The granules in the fibroblasts from the patient were clustered in the perinuclear area, whereas the granules in the fibroblasts from the mother and control were dispersed throughout the cytoplasm. After incubation with ascorbic acid, the clustered granules in the fibroblasts of the Chediak-Higashi syndrome showed a tendency to spread throughout the cytoplasm. The distribution pattern of the granules was studied by quantitative morphology.

References

Nov 4, 1976·The New England Journal of Medicine·L A BoxerR L Baehner
Jul 1, 1976·Pediatric Research·K AbeM Ishikawa
Jul 8, 1967·Lancet·B S Danes, A G Bearn
Nov 1, 1974·Pediatric Research·U N Wiesmann, N N Herschkowitz
Feb 1, 1972·Annals of Internal Medicine·S M Wolff
Mar 28, 1974·The New England Journal of Medicine·T P Stossel
Dec 1, 1971·The Journal of Clinical Investigation·R A Clark, H R Kimball
Jan 20, 1972·The New England Journal of Medicine·T P StosselM Vaughan

Citations

Jul 1, 1990·American Journal of Medical Genetics·M M Kahraman, D J Prieur
May 1, 1986·Journal of Clinical Immunology·F MerinoP Ramirez-Duque
Nov 1, 1993·In Vitro Cellular & Developmental Biology. Animal·J B GowT A Lyerla
Mar 1, 1988·Veterinary Pathology·J D Penner, D J Prieur

Related Concepts

Magnorbin
Chediak-Higashi Syndrome
Cytoplasmic Granules
Fibroblasts
Lysosomes
Skin

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