Maroteaux-Lamy Syndrome

Maroteaux-Lamy syndrome, also known as mucopolysaccharidosis type 6 (MPS6), is a metabolic disease, more specifically a lysosomal storage disease, with progressive multisystem involvement, due to the lack of activity of arylsulfatase B.

The symptoms are very broad and lead to death depending on the speed of the progressive course in the second to fifth decade of life. Affected persons are usually not restricted in their intellectual property.

The reason for this is the accumulated mutations on the ARSB gene, which results in a reduction or discontinuation of the degradation of dermatan sulfate and chondriotine sulfate. The disease is inherited as an autosomal recessive trait. 

Maroteaux-Lamy syndrome, also known as mucopolysaccharidosis type 6 (MPS6), is a metabolic disease, more specifically a lysosomal storage disease, with progressive multisystem involvement due to the deficiency of arylsulfatase B activity. The symptoms are very broad and lead to death, depending on the rate of progressive progression in the 2nd to 5th year of age. Affected persons are usually unaffected in their intellectual property. The causes are the mutations in the ARSB gene, resulting in a reduction or termination of the degradation of dermatan sulphate and chondriotin sulphate. The disease is inherited as an autosomal recessive trait. & Nbsp;