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  2. GM2 (ganglioside) - Wikipedia

    en.wikipedia.org/wiki/GM2_(ganglioside)

    In organic chemistry, GM2 is a type of ganglioside. G refers to ganglioside, the M is for monosialic (as in it has one sialic acid), and 2 refers to the fact that it was the second monosialic ganglioside discovered. It is associated with GM2 gangliosidoses such as Tay–Sachs disease. [1]

  3. Ganglioside - Wikipedia

    en.wikipedia.org/wiki/Ganglioside

    The functions of gangliosides as specific determinants suggest its important role in the growth and differentiation of tissues as well as in carcinogenesis. It has been found that tumor formation can induce the synthesis of a new complement of ganglioside, and very low concentrations of a specific ganglioside can induce differentiation of ...

  4. GM2A - Wikipedia

    en.wikipedia.org/wiki/GM2A

    Function [ edit ] The protein encoded by this gene is a small glycolipid transport protein which acts as a substrate specific co-factor for the lysosomal enzyme β-hexosaminidase A . β-hexosaminidase A, together with GM2 ganglioside activator, catalyzes the degradation of the ganglioside GM2 , and other molecules containing terminal N-acetyl ...

  5. GM2 gangliosidoses - Wikipedia

    en.wikipedia.org/wiki/GM2_gangliosidoses

    GM2-gangliosidosis, AB variant is a rare, autosomal recessive metabolic disorder that causes progressive destruction of nerve cells in the brain and spinal cord. Mutations in the GM2A gene cause AB variant. The GM2A gene provides instructions for making a protein called the GM2 activator.

  6. B4GALNT1 - Wikipedia

    en.wikipedia.org/wiki/B4GALNT1

    GM2 and GD2 gangliosides are sialic acid-containing glycosphingolipids. GalNAc-T is the enzyme involved in the biosynthesis of G(M2) and G(D2) glycosphingolipids. GalNAc-T catalyzes the transfer of GalNAc into G(M3) and G(D3) by a beta-1,4 linkage, resulting in the synthesis of G(M2) and G(D2), respectively. [6]

  7. Sandhoff disease - Wikipedia

    en.wikipedia.org/wiki/Sandhoff_disease

    Sandhoff disease is a lysosomal genetic, lipid storage disorder caused by the inherited deficiency to create functional beta-hexosaminidases A and B. [1] [2] These catabolic enzymes are needed to degrade the neuronal membrane components, ganglioside GM2, its derivative GA2, the glycolipid globoside in visceral tissues, [1] and some oligosaccharides.

  8. HEXA - Wikipedia

    en.wikipedia.org/wiki/HEXA

    The HEXA gene is a protein encoding gene that codes for the lysosomal enzyme beta-hexosaminidase. This enzyme, combined with the GM2 activator protein, is responsible for the breakdown of ganglioside GM2 within the lysosome. Defects in the HEXA gene, however, prevent this degradation, leading to a buildup of toxins in brain and spinal cord cells.

  9. (N-acetylneuraminyl)-galactosylglucosylceramide N ...

    en.wikipedia.org/wiki/(N-acetylneuraminyl)-ga...

    (N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase (EC 2.4.1.92, uridine diphosphoacetylgalactosamine-ganglioside GM3 ...