Defective phagolysosome is feature of?
Correct Answer: Chediak - Higashi - syndrome
Description: Inherited defects in phagolysosome function. One such disorder is Chediak-Higashi syndrome, Autosomal recessive condition Defective fusion of phagosomes and lysosomes, resulting in defective phagocytes function and susceptibility to infections. The main leukocyte abnormalities are:- Neutropenia Defective degranulation Delayed microbial killing The gene associated with this disorder encodes a large cytosolic protein called LYST, which is believed to regulate lysosomal trafficking.
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Pathology
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