What is MPS VI?

What is MPS VI?

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). Skeletal abnormalities are also common in this condition.

What is MPS in animals?

MPS, a group of inherited metabolic disorders, is due to defects in a series of catabolic enzymes, which results in multisystemic accumulation of improperly degraded glycosaminoglycans (GAGs). The undegraded GAGs usually leak out of the cells and can be detected in the urine, which serves as a screening test.

What is MPS VI in dogs?

Mucopolysaccharidosis type VI should be considered as a differential diagnosis for young dogs and cats with stunted growth, progressive skeletal malformations, and leukocyte inclusions, particularly in miniature pinschers, miniature schnauzers, miniature poodles, and Siamese and domestic shorthair cats.

What is MPS life expectancy?

The life expectancy of these individuals is 10 to 20 years. Individuals with mild MPS II also have a shortened lifespan, but they typically live into adulthood and their intelligence is not affected. Heart disease and airway obstruction are major causes of death in people with both types of MPS II.

What does MPS mean for dogs?

Mucopolysaccharidosis I (MPS I) is a rare disease that occurs in dogs and humans due to a deficiency of the lysosomal enzyme, alpha-L-iduronidase. Humans affected with MPS I experience mild to severe clinical signs in facial features, skeletal changes, cognitive decline, and heart, liver, and respiratory disease.

What is cat MPS?

Mucopolysaccharidosis (MPS) is a group of lysosomal storage diseases that are inherited as an autosomal recessive trait. These are classified as: MPS I – α-L-iduronidase deficiency, reported in domestic shorthair cats.

How long do dogs with MPS live?

Clinical signs of MPS I affected dogs are analogous to the human intermediate form, which, again, include, skeletal deterioration, joint laxity, hepatosplenomegaly, heart disease, impaired vision, and a lifespan of less than 3 years [24,39].

What is MPS IIIB dog?

Mucopolysaccharidosis type IIIB (MPS IIIB; Sanfilippo syndrome B) is an autosomal recessive lysosomal storage disorder caused by the deficiency of alpha-N-acetylglucosaminidase activity, leading to increased levels of nondegraded heparan sulfate (HS).