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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 ]
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 ...
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.
Diagram showing the development of different blood cells from haematopoietic stem cell to mature cells. Haematopoiesis (/ h ɪ ˌ m æ t ə p ɔɪ ˈ iː s ɪ s, ˌ h iː m ə t oʊ-, ˌ h ɛ m ə-/; [1] [2] from Ancient Greek αἷμα (haîma) 'blood' and ποιεῖν (poieîn) 'to make'; also hematopoiesis in American English, sometimes h(a)emopoiesis) is the formation of blood cellular ...
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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 ...