Pertussis toxin they are formed from two subunits. The "A" subunit possesses enzyme activity, and is transferred to the host cell following a conformational change in the membrane-bound transport "B" subunit. Pertussis toxin is an exotoxin with six subunits (named "S1" through "S5"—each complex contains two copies of "S4"). The subunits are arranged in "A-B" structure: the "A" component is enzymatically active and is formed from the S1 subunit, while the "B" component is the receptor-binding portion and is made up of subunits S2–S5. The subunits are encoded by "ptx" genes encoded on a large PT operon that also includes additional genes
Schindler disease Schindler disease Schindler disease, also known as Kanzaki disease and alpha-N-acetylgalactosaminidase deficiency is a rare disease found in humans. This lysosomal storage disorder is caused by a deficiency in the enzyme alpha-NAGA (alpha-N-acetylgalactosaminidase), attributable to mutations in the NAGA gene on chromosome 22, which leads to excessive lysosomal accumulation of glycoproteins. A deficiency of the alpha-NAGA enzyme leads to an accumulation of glycosphingolipids throughout the body. This accumulation of sugars gives rise to the clinical features associated with this disorder. Schindler disease is an autosomal recessive disorder, meaning that one must inherit an abnormal allele from both parents in order