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More than 60 gangliosides are known, which differ from each other mainly in the position and number of NANA residues. It is a component of the cell plasma membrane that modulates cell signal transduction events, and appears to concentrate in lipid rafts [2] [3]. Recently, gangliosides have been found to be highly important molecules in ...
The numbering is based on its relative mobility in electrophoresis among other monosialic gangliosides. [1] Its structure can be condensed to NANA - Gal - Glc - ceramide . GM3 is the most common membrane-bound glycosphingolipid in tissues, composed of three monosaccharide groups attached to a ceramide backbone. [ 2 ]
Myeloid tissue, in the bone marrow sense of the word myeloid (myelo-+ -oid), is tissue of bone marrow, of bone marrow cell lineage, or resembling bone marrow, and myelogenous tissue (myelo-+ -genous) is any tissue of, or arising from, bone marrow; in these senses the terms are usually used synonymously, as for example with chronic myeloid ...
The GM1 gangliosidoses, usually shortened to GM1, are gangliosidoses caused by mutation in the GLB1 gene resulting in a deficiency of beta-galactosidase.The deficiency causes abnormal storage of acidic lipid materials in cells of the central and peripheral nervous systems, but particularly in the nerve cells, resulting in progressive neurodegeneration.
Bone marrow is a semi-solid tissue found within the spongy (also known as cancellous) portions of bones. [2] In birds and mammals, bone marrow is the primary site of new blood cell production (or haematopoiesis). [3] It is composed of hematopoietic cells, marrow adipose tissue, and supportive stromal cells.
The secreted toxin attaches to the surface of the host mucosa cell by binding to GM1 gangliosides. GM1 consists of a sialic acid-containing oligosaccharide covalently attached to a ceramide lipid. The A1 subunit of this toxin will gain entry to intestinal epithelial cells with the assistance of the B subunit via the GM1 ganglioside receptor.
Haematopoietic stem cell transplantation (HSCT) is the transplantation of multipotent haematopoietic stem cells, usually derived from bone marrow, peripheral blood, or umbilical cord blood. [ 10 ] [ 11 ] [ 12 ] It may be autologous (the patient's own stem cells are used), allogeneic (the stem cells come from a donor) or syngeneic (from an ...
This enzyme catalyzes the biodegradation of fatty acid derivatives known as gangliosides. [1] The diseases are better known by their individual names: Tay–Sachs disease, AB variant, and Sandhoff disease. Beta-hexosaminidase is a vital hydrolytic enzyme, found in the lysosomes, that breaks down lipids. When beta-hexosaminidase is no longer ...