Maroteaux-Lamy syndrome - определение. Что такое Maroteaux-Lamy syndrome
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Что (кто) такое Maroteaux-Lamy syndrome - определение

LYSOSOMAL STORAGE DISEASE
Mucopolysaccharidosis type VI Maroteaux-Lamy - severe, intermediate; Maroteaux-Lamy syndrome; Mucopolysaccharidosis vi; Maroteaux-Lamy Syndrome; MPS VI; Maroteaux Lamy disease; Mucopolysaccharidosis VI; Mucopolysaccharidosis type VI; Mucopolysaccharidosis VI (Maroteaux-Lamy); MPS6; MPS 6; Maroteaux-Lamy; Mucopolysaccharidosis type 6; Polydystrophic dwarfism
  • Corneal clouding visible in the eye of a 30-year-old male with MPS VI
  • Dermatan sulfate is one of the GAGs that builds up in the tissues of people with MPS-VI.
  • [[Isabel Bueso]] in 2019
  • A slowly-progressing female patient in her 20s, showing few physical abnormalities

André Lamy         
FILM PRODUCER
Andre Lamy; André Lamy (film producer)
André Lamy (19 July 1932 – 2 May 2010) was a Canadian film producer, who served as Canada's Government Film Commissioner from 1975 until 1979. In this position he was the Chairman of the National Film Board of Canada (NFB).
Étienne Lamy         
  • Étienne Lamy.
FRENCH AUTHOR (1845-1919)
Etienne Lamy
Étienne Marie Victor Lamy (2 June 1845, in Cize, Jura – 9 January 1919) was a French author. He was educated at the College Stanislas and became a doctor of law in 1870.
Joseph Alfred Lamy         
FRENCH LUTHIER
Lamy, Joseph Alfred; Lamy (bow makers); Alfred Joseph Lamy
Joseph Alfred Lamy (père) (8 September 1850 – 1919), was an important French archetier (bow maker) of the early twentieth century known as Lamy Père.

Википедия

Maroteaux–Lamy syndrome

Maroteaux–Lamy syndrome, or Mucopolysaccharidosis Type VI (MPS-VI), is an inherited disease caused by a deficiency in the enzyme arylsulfatase B (ARSB). ASRB is responsible for the breakdown of large sugar molecules called glycosaminoglycans (GAGs, also known as mucopolysaccharides). In particular, ARSB breaks down dermatan sulfate and chondroitin sulfate. Because people with MPS-VI lack the ability to break down these GAGs, these chemicals build up in the lysosomes of cells. MPS-VI is therefore a type of lysosomal storage disease.