Maroteaux–Lamy syndrome, or mucopolysaccharidosis Type VI (MPS-VI), is an inherited disease caused by a deficiency in the enzyme arylsulfatase B (ARSB). [3] ASRB is responsible for the breakdown of large sugar molecules called glycosaminoglycans (GAGs, also known as mucopolysaccharides).

Mucopolysaccharidosis type VI (MPS VI), also known as Maroteaux-Lamy syndrome, is a progressive condition that causes many tissues and organs to enlarge, become inflamed or scarred, and eventually waste away (atrophy).

Mucopolysarcharidosis type VI (MPS VI) is a rare, inherited disorder that is classified as a lysosomal storage disorder (LSD) in which a genetic variation disrupts the normal activity of lysosomes in human cells. MPS VI also known as Maroteaux-Lamay syndrome.

Mucopolysaccharidosis type VI (MPS VI), or Maroteaux–Lamy syndrome (MIM# 253200), is a rare, inherited, autosomal recessive metabolic disorder caused by low to absent activity of the lysosomal enzyme N-acetylgalactosamine 4-sulfatase (arylsulfatase B; ASB; EC 3.1.6.12) that catalyzes one of the steps of degradation of the glycosaminoglycans ...

Jul 16, 2020 · Maroteaux-Lamy syndrome (mucopolysaccharidosis type VI; MPS VI) is a rare genetic disorder characterized by complete or partial lack of activity of the enzyme arylsulfatase B (also called N-acetylgalactosamine-4-sulfatase), encoded by the ARSB gene.

Jul 19, 2024 · MPS VI (also known as Maroteaux-Lamy syndrome) is caused by the deficient enzyme N-acetylgalactosamine 4-sulfatase. MPS VI has a wide range of symptoms. Some individuals experience a few mild symptoms, while others develop more severe symptoms.

Mucopolysaccharidosis (MPS) VI or Maroteaux-Lamy syndrome (253200) is an autosomal recessive lysosomal storage disorder caused by deficiency in N -acetylgalactosamine-4-sulfatase (arylsulfatase B).

What is MPS VI? MPS VI, known as Maroteaux-Lamy disease, is one of the mucopolysaccharide storage diseases. MPS VI was first identified by Dr Maroteaux and Dr Lamy in 1963.

MPS VI (Maroteaux-Lamy syndrome) is caused by a recessive gene. There is a one in four chance with every pregnancy that the child will inherit the defective gene from each carrier parent and will be affected with the disease.

Mucopolysaccharidosis type 6 (MPS 6) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B (ASB) leading to the accumulation of dermatan sulfate.