GM2 activator protein

a sphingolipid activator protein that binds GM2 ganglioside and related glycosphingolipids and presents them to hexosaminidase A for cleavage; it is necessary for enzyme activity. Deficiency results in GM2 gangliosidosis, AB variant.

Medical dictionary. 2011.

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  • Ganglioside GM2 activator protein — is (GM2 AP) is a lipid transfer protein that stimulates the enzymatic processing of gangliosides, and also T cell activation through lipid presentation.This protein binds molecules of ganglioside GM2, extracts them from membranes, and presents… …   Wikipedia

  • sphingolipid activator protein — (SAP) any of a group of nonenzyme lysosomal proteins that stimulate the actions of specific lysosomal hydrolases by binding and solubilizing their sphingolipid substrates; it includes the saposins and GM2 activator protein (qq.v.) …   Medical dictionary

  • GM2-Aktivator — Bändermodell nach PDB  …   Deutsch Wikipedia

  • GM2 (ganglioside) — GM2 is a type of ganglioside.It is associated with GM2 gangliosidoses (for example, Tay Sachs disease.)cite journal |author=Guetta E, Peleg L |title=Rapid detection of fetal mendalian disorders: tay sachs disease |journal=Methods Mol. Biol.… …   Wikipedia

  • GM2 g. AB variant — a GM2 gangliosidosis caused by mutation in the GM2Agene (locus: 5q31.3 q33.1), which encodes GM2 activator protein, a sphingolipid activator protein necessary for hexosaminidase A activity; this variant is clinically identical to infantile Tay… …   Medical dictionary

  • GM2-gangliosidosis, AB variant — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 32644 ICD10 = ICD9 = ICDO = OMIM = 272750 MedlinePlus = eMedicineSubj = ped eMedicineTopic = 3016 MeshID = D049290 GM2 gangliosidosis, AB variant is a rare, autosomal recessive metabolic… …   Wikipedia

  • GM2 gangliosidosis — any of a group of lysosomal storage diseases characterized by abnormal accumulation of ganglioside GM2 and related glycoconjugates, due to deficiency of activity of one or more hexosaminidase isozymes or of an activator protein necessary for… …   Medical dictionary

  • GM2A — GM2 ganglioside activator, also known as GM2A, is a human gene. PBB Summary section title = summary text = The protein encoded by this gene is a small glycolipid transport protein which acts as a substrate specific co factor for the lysosomal… …   Wikipedia

  • Hexosaminidase — β N acetylhexosaminidase Hexosaminidase A (Hex A) Identifiers EC number 3.2.1.52 …   Wikipedia

  • Prosaposin — PDB rendering based on 1m12 …   Wikipedia

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