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Sly syndrome information


Sly syndrome
Other namesβ-glucuronidase deficiency, β-glucuronidase deficiency mucopolysaccharidosis,
Sly syndrome has an autosomal recessive pattern of inheritance.
SpecialtyEndocrinology Edit this on Wikidata

Sly syndrome, also called mucopolysaccharidosis type VII (MPS-VII), is an autosomal recessive lysosomal storage disease caused by a deficiency of the enzyme β-glucuronidase. This enzyme is responsible for breaking down large sugar molecules called glycosaminoglycans (AKA GAGs, or mucopolysaccharides). The inability to break down GAGs leads to a buildup in many tissues and organs of the body. The severity of the disease can vary widely.[1]

  1. ^ "Mucopolysaccharidosis type VII". United States National Library of Medicine. 25 June 2019. Retrieved 2 July 2019.

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Sly syndrome

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type II (Hunter syndrome). Vestronidase alfa (Mepsevii) is a recombinant human lysosomal beta glucuronidase for MPS VII (Sly syndrome) approved in the...

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Sly

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Look up sly in Wiktionary, the free dictionary. Sly or SLY may refer to: Sly Stone (born 1943), American musician in Sly and the Family Stone Sylvester...

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Mucopolysaccharidosis type 7 Sly syndrome Mucopolysaccharidosis type I Hurler syndrome Mucopolysaccharidosis type I Hurler/Scheie syndrome Mucopolysaccharidosis...

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Pectus carinatum

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homocystinuria, osteogenesis imperfecta, multiple lentigines syndrome (LEOPARD syndrome), Sly syndrome (mucopolysaccharidosis type VII), and scoliosis. In about...

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Morquio syndrome) MPS VI Mucopolysaccharoidosis type VI (see Maroteaux–Lamy syndrome) MPS VII Mucopolysaccharoidosis type VII (see Sly syndrome) MPX Monkeypox...

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Vestronidase alfa

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alfa, sold under brand name Mepsevii, is a drug for the treatment of Sly syndrome. It is a recombinant form of the human enzyme beta-glucuronidase. It...

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sulfate deficiency) Sanfilippo syndrome (MPS III) Morquio syndrome (MPS IV) Maroteaux–Lamy syndrome (MPS VI) Sly syndrome (MPS VII) Glycoproteinoses Mucolipidosis...

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Lysosomal storage disease

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MPS IVA (Type A) MPS IVB (Type B) Type VI (Maroteaux–Lamy syndrome) Type VII (Sly syndrome) Type IX (hyaluronidase deficiency) Mucolipidosis Type I (sialidosis)...

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Sanfilippo syndrome Scheie syndrome (mucopolysaccharidosis type I S) Secondary cutaneous amyloidosis Secondary systemic amyloidosis Sialidosis Sly syndrome...

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mitochondrial heredity William S. Sly (born 1932), US biochemical geneticist, mucopolysaccharidosis type VII (Sly syndrome) Cedric A. B. Smith (1917–2002)...

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Chromosome 7

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metabolic condition mucopolysaccharidosis type VII (MPS VII), also known as Sly syndrome. Burosumab (KRN-23; brand name Crysvita) was approved in 2018 by the...

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Renal tubular acidosis

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Shah GN, Bonapace G, Hu PY, Strisciuglio P, Sly WS (September 2004). "Carbonic anhydrase II deficiency syndrome (osteopetrosis with renal tubular acidosis...

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Contiguous gene syndrome

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Cytogenetics of Contiguous Gene Syndromes: Mechanisms and Consequences of Gene Dosage Imbalance". In Scriver, C.W.; Beaudet, A.L.; Sly, W.S.; et al. (eds.). Metabolic...

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The Taming of the Shrew

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tinker named Christopher Sly into believing he is actually a nobleman himself. The nobleman then has the play performed for Sly's diversion. The main plot...

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Atopy

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P; Knight, Julian C; Gupta, Ramneek; Thompson, Philip J; Holt, Patrick; Sly, Peter; Hirschhorn, Joel N; Blekic, Mario; Weidinger, Stephan; Hakonarsson...

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also called hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, is a rare autosomal recessive urea cycle disorder affecting the enzyme...

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(1985). He later appeared in the Dentyne gum commercials where he would slyly utter the "Time to walk the dog" catch phrase. Morrow played the lead role...

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List of ZX Spectrum games

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Ivan Horn, Matthew Cannon Orion Pictures Corporation/Tobor Productions 1990 Sly Spy Ocean Software Software Creations (Dean Belfield, John P. Tatlock, Simon...

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Digital Underground

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More" with Luniz. The album also contains an early performance from rapper Sly Boogy, while he was still a member of the Black Spooks, who appeared on the...

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"131. X-Linked Adrenoleukodystrophy". In Scriver, C.W.; Beaudet, A.L.; Sly, W.S.; Valle, D.; Childs, B.; Kinzler, K.W.; Vogelstein, B. (eds.). Metabolic...

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Carbonic anhydrase 12

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