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| Similar words: G(M1) Ganglioside G(M2) Ganglioside G(M3) Ganglioside | |
| G(M3) Ganglioside Hematoside | Sialyllactosylceramide | Ganglioside GM3 | II3NeuAcLacCer | Sialyl Lactosylceramide | GM3, Ganglioside | Lactosylceramide, Sialyl A ganglioside present in abnormally large amounts in the brain and liver due to a deficient biosynthetic enzyme, G(M3):UDP-N-acetylgalactosaminyltransferase. Deficiency of this enzyme prevents the formation of G(M2) ganglioside from G(M3) ganglioside and is the cause of an anabolic sphingolipidosis. | |
| G(M2) Ganglioside Tay-Sachs Disease Ganglioside | Ganglioside GM2 | GM2, Ganglioside | Ganglioside, Tay-Sachs Disease | Tay Sachs Disease Ganglioside A glycosphingolipid that accumulates due to a deficiency of hexosaminidase A or B (BETA-N-ACETYLHEXOSAMINIDASE), or GM2 activator protein, resulting in GANGLIOSIDOSES, heredity metabolic disorders that include TAY-SACHS DISEASE and SANDHOFF DISEASE. | |
| Gangliosidoses Ganglioside Storage Diseases | Ganglioside Storage Disorders | Gangliosidosis | Ganglioside Storage Disease | Ganglioside Storage Disorder | Storage Disease, Ganglioside | Storage Diseases, Ganglioside | Storage Disorder, Ganglioside A group of often fatal inherited diseases marked by the accumulation of GANGLIOSIDES in LYSOSOMES secondary to enzymatic deficiency states. Gangliosidoses include TAY-SACHS DISEASE; GANGLIOSIDOSIS GM1; GANGLIOSIDOSES GM2; and SANDHOFF DISEASE; which share the infantile or childhood onset of CENTRAL NERVOUS SYSTEM deterioration. (Menkes, Textbook of Child Neurology, 5th ed, pp89-97) | |
| G(M1) Ganglioside Monosialosyl Tetraglycosyl Ceramide | G(A(2)) Ganglioside | GA(2) Ganglioside | GM1a Monosialoganglioside | Ceramide, Monosialosyl Tetraglycosyl | Monosialoganglioside, GM1a | Tetraglycosyl Ceramide, Monosialosyl A specific monosialoganglioside that accumulates abnormally within the nervous system due to a deficiency of GM1-b-galactosidase, resulting in GM1 gangliosidosis. | |
| Gangliosides Sialoglycosphingolipids A subclass of ACIDIC GLYCOSPHINGOLIPIDS. They contain one or more sialic acid (N-ACETYLNEURAMINIC ACID) residues. Using the Svennerholm system of abbrevations, gangliosides are designated G for ganglioside, plus subscript M, D, or T for mono-, di-, or trisialo, respectively, the subscript letter being followed by a subscript arabic numeral to indicated sequence of migration in thin-layer chromatograms. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1997) | |
| N-Acylneuraminate Cytidylyltransferase Acylneuraminate Cytidylyltransferase | CMP Sialate Pyrophosphorylase | CMP Sialate Synthase | CMP-N-Acetylneuraminic Acid Synthetase | CMP-Sialic Acid Synthetase | Cytidine 5' | -Monophosphosialic Acid Synthetase | Sialate Synthase, CMP An enzyme that forms CMP-acylneuraminic acids, which donate the N-acylneuraminic acid residues to the terminal sugar residue of a ganglioside or glycoprotein. EC 2.7.7.43. | |
| Gangliosidoses GM2 G(M2) Gangliosidoses | GM2 Protein Activator Deficiency Disease | Gangliosidosis GM2, AB Variant | Gangliosidosis GM2, Type AB | AB Variant Gangliosidosis GM2 | Deficiency Disease, GM2 Protein Activator | GM2 Activator Deficiency Disease Inherited diseases characterized by the accumulation of G(M2) GANGLIOSIDE in central nervous system lysosomes and variably in other tissues. Subtypes include TAY-SACHS DISEASE and SANDHOFF DISEASE, as well as an AB variant of BETA-N-ACETYLHEXOSAMINIDASE deficiency and an adult onset form of GM2 Gangliosidosis. | |
| Cytidine Monophosphate N-Acetylneuraminic Acid CMP Acetylneuraminic Acid | CMP-NANA | CMP-Sialic Acid | Cytidine Monophosphate N Acetylneuraminic Acid | Acetylneuraminic Acid, CMP | Acid, CMP Acetylneuraminic | Acid, CMP-Sialic | CMP Sialic Acid A nucleoside monophosphate sugar which donates N-acetylneuraminic acid to the terminal sugar of a ganglioside or glycoprotein. | |
| Tay-Sachs Disease G(M2) Gangliosidosis, Type I | GM2 Gangliosidosis, Type I | Gangliosidosis G(M2), Type I | Gangliosidosis GM2, Type I | Hexosaminidase A Deficiency Disease | Deficiency Disease Hexosaminidase A | Tay Sachs Disease An autosomal recessive inherited gangliosidosis characterized by the onset in the first 6 months of life of an exaggerated startle response, delay in psychomotor development, hypotonia (followed by spasticity), visual loss, and a macular cherry red spot. Hexosaminidase A (see BETA-N-ACETYLHEXOSAMINIDASE) is deficient, leading to the accumulation of GM2 ganglioside in neurons of the central nervous system and retina. This condition is strongly associated with Askenazic Jewish ancestory. (Menkes, Textbook of Pediatric Neurology, 5th ed pp89-96) | |
| Mucolipidoses Cherry Red Spot Myoclonus Syndrome | Ganglioside Sialidase Deficiency Disease | I-Cell Disease | Lipomucopolysaccharidosis | Mucolipidosis | Myoclonus Cherry Red Spot Syndrome | Pseudo-Hurler Polydystrophy | Sialidosis | Inclusion Cell Disease A group of inherited metabolic diseases characterized by the accumulation of excessive amounts of acid mucopolysaccharides, sphingolipids, and/or glycolipids in visceral and mesenchymal cells. Abnormal amounts of sphingolipids or glycolipids are present in neural tissue. Mental retardation and skeletal changes, most notably dysostosis multiplex, occur frequently. (From Joynt, Clinical Neurology, 1992, Ch56, pp36-7) | |
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