Defective phagolysosome is feature of?: (A) Chronic Granulomatous Disease, (B) Chediak - Higashi - syndrome, (C) Ataxia telangiectasia, (D) Fanconi Anemia
Answer is B. 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.