Abstract
A new indirect immunofluorescent technique was employed in studies of a 1 y/o M with CHS to delineate possible functional relationships between assemblage of MTs & PMN motility, morphology & surface distributions of adhesion or lectin binding sites. CH PMNs exposed to gradients of fMLP demonstrated diminished orientation/migration (p <.001) but normal numbers of MT/PMNs [34±5 (CH), 36±7 (control)] & normal MT lengths - [CHS; 6.9±2.0 μm (PBS) → 10.1±3 μm (fMLP) vs control; 7.1±2 μm (PBS) → 10.9±3.1 μm (fMLP)]. MTs of CH & normal PMNs were equally susceptible to depolymerization by colchicine (μM) & their reassemblage following fMLP stimuli was normal. Shape change by CH PMNs stimulated in suspension with fMLP (2 nM, 5 min) was diminished [mean % bipolar + uropod forms = 30±7 (CHS), 92±6 (controls), p <.001]. Colchicine (μM) promoted significantly less bipolar shape change of CH PMNs (10±6%) as compared to normal PMNs (55±10%) (p <.001). Conditions promoting a redistribution of surface binding sites for albumin coated latex beads (ACLB) to the cell uropod in normal PMNs [colchicine (μM) or fMLP (0.1 nM, 10 nM)] failed to redistribute (cap) ACLB binding sites of CH PMNs. In contrast, CH PMNs demonstrated enhanced spontaneous concanavalin A cap** [55±7% (CH), 8±6% (control), p <.001] or cap** following colchicine (μM) preincubation [84±11% (CH), 61±10% (control)]. Thus, abnormalities of PMN mobility, shape change & surface distributions of lectin or ACLB binding sites mediated by chemotactic factors are unrelated to pathologic MT assembly.
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Anderson, D., Hughes, B., Wible, L. et al. NORMAL MICROTUBULE (MT) ASSEMBLY BY CHEDIAK-HIGASHI (CH) PMNs. Pediatr Res 18 (Suppl 4), 252 (1984). https://doi.org/10.1203/00006450-198404001-00952
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DOI: https://doi.org/10.1203/00006450-198404001-00952
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