Aspartylglucosamine

Last updated
Aspartylglucosamine
Aspartylglucosamine.png
Names
IUPAC name
(2S)-4-[[(2R,3R,4R,5S,6R)-3-Acetamido-4,5-dihydroxy-6-(hydroxymethyl)oxan-2-yl]amino]-2-amino-4-oxobutanoic acid
Identifiers
3D model (JSmol)
ChEBI
ChemSpider
MeSH N-acetylglucosaminylasparagine
PubChem CID
UNII
  • InChI=1S/C12H21N3O8/c1-4(17)14-8-10(20)9(19)6(3-16)23-11(8)15-7(18)2-5(13)12(21)22/h5-6,8-11,16,19-20H,2-3,13H2,1H3,(H,14,17)(H,15,18)(H,21,22)/t5-,6+,8+,9+,10+,11+/m0/s1 Yes check.svgY
    Key: YTTRPBWEMMPYSW-HRRFRDKFSA-N Yes check.svgY
  • InChI=1/C12H21N3O8/c1-4(17)14-8-10(20)9(19)6(3-16)23-11(8)15-7(18)2-5(13)12(21)22/h5-6,8-11,16,19-20H,2-3,13H2,1H3,(H,14,17)(H,15,18)(H,21,22)/t5-,6+,8+,9+,10+,11+/m0/s1
    Key: YTTRPBWEMMPYSW-HRRFRDKFBT
  • CC(=O)N[C@@H]1[C@H]([C@@H]([C@H](O[C@H]1NC(=O)C[C@@H](C(=O)O)N)CO)O)O
Properties
C12H21N3O8
Molar mass 335.31 g/mol
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
X mark.svgN  verify  (what is  Yes check.svgYX mark.svgN ?)

Aspartylglucosamine [1] is a derivative of aspartic acid.

Levels are elevated in aspartylglucosaminuria. [2]

Related Research Articles

<span class="mw-page-title-main">Charles II of Spain</span> King of Spain from 1665 to 1700

Charles II of Spain was King of Spain from 1665 to 1700. The last monarch from the House of Habsburg, which had ruled Spain since 1516, he died without children, leading to a European conflict over his successor.

<span class="mw-page-title-main">Lysosomal storage disease</span> Medical condition

Lysosomal storage diseases are a group of over 70 rare inherited metabolic disorders that result from defects in lysosomal function. Lysosomes are sacs of enzymes within cells that digest large molecules and pass the fragments on to other parts of the cell for recycling. This process requires several critical enzymes. If one of these enzymes is defective due to a mutation, the large molecules accumulate within the cell, eventually killing it.

Agu is a surname and given name.

<span class="mw-page-title-main">Leukodystrophy</span> Group of disorders characterised by degeneration of white matter in the brain

Leukodystrophies are a group of, usually, inherited disorders, characterized by degeneration of the white matter in the brain. The word leukodystrophy comes from the Greek roots leuko, "white", dys, "abnormal" and troph, "growth". The leukodystrophies are caused by imperfect growth or development of the glial cells which produce the myelin sheath, the fatty insulating covering around nerve fibers. Leukodystrophies may be classified as hypomyelinating or demyelinating diseases, respectively, depending on whether the damage is present before birth or occurs after. While all leukodystrophies are the result of genetic mutations, other demyelinating disorders have an autoimmune, infectious, or metabolic etiology.

<span class="mw-page-title-main">Aspartylglucosaminuria</span> Medical condition

Aspartylglucosaminuria (AGU) is an inherited disease that is characterized by a decline in mental functioning, accompanied by an increase in skin, bone and joint issues.

<span class="mw-page-title-main">Aspartylglucosaminidase</span> Protein-coding gene in the species Homo sapiens

N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase is an enzyme that in humans is encoded by the AGA gene.

<span class="mw-page-title-main">Congenital disorders of amino acid metabolism</span> Medical condition

Inborn errors of amino acid metabolism are inherited metabolic disorders that impair the synthesis and degradation of amino acids.

<span class="mw-page-title-main">Galactosialidosis</span> Rare disease

Galactosialidosis, also known as neuraminidase deficiency with beta-galactosidase deficiency, is a genetic lysosomal storage disease. It is caused by a mutation in the CTSA gene which leads to a deficiency of enzymes β-galactosidase and neuraminidase. This deficiency inhibits the lysosomes of cells from functioning properly, resulting in the accumulation of toxic matter within the cell. Hallmark symptoms include abnormal spinal structure, vision problems, coarse facial features, hearing impairment, and intellectual disability. Because galactosialidosis involves the lysosomes of all cells, it can affect various areas of the body, including the brain, eyes, bones, and muscles. Depending on the patient's age at the onset of symptoms, the disease consists of three subtypes: early infantile, late infantile, and juvenile/adult. This condition is considered rare, with most cases having been in the juvenile/adult group of patients.

References

  1. ↑ PubChem. "N-aspartylglucosamine". pubchem.ncbi.nlm.nih.gov. Retrieved 2023-01-13.
  2. ↑ "Aspartylglucosaminuria i". ISMRD — The International Advocate for Glycoprotein Storage Diseases.


This page is based on this Wikipedia article
Text is available under the CC BY-SA 4.0 license; additional terms may apply.
Images, videos and audio are available under their respective licenses.