Disulfide Connectivity of Velaglucerase: Difference between revisions

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Joel L. Sussman (talk | contribs)
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<applet load='1ogs' size='[450,338]' frame='true' align='right'
<applet load='1ogs' size='[450,338]' frame='true' align='right'
caption='Human beta-glucocerebrosidase (1ogs)' scene='User:Adriana_Kita/Sandbox_1/Gcase_whole/5'/>
caption='Human beta-glucocerebrosidase ([[1ogs]])' scene='User:Adriana_Kita/Sandbox_1/Gcase_whole/5'/>


Human beta-glucocerebrosidase, also known as glucoceremidase, GCase, and velaglucerase, is an enzyme that is commonly found to be deficient in patients with Gaucher's disease.  
Human beta-glucocerebrosidase, also known as glucoceremidase, GCase, and velaglucerase, is an enzyme that is commonly found to be deficient in patients with Gaucher's disease. <ref group="xtra">PMID:12792654</ref><ref group="xtra">PMID:17524049</ref><ref group="xtra">PMID:19741058</ref>
 
Currently, there are three drugs on the market to treat this disease. One being Genzyme's Cerezyme, Protalix's Taliglucerase and SHIRE's VPRIV.  
Currently, there are two drugs on the market to treat this disease. One being Genzyme's Cerezyme and the other SHIRE's VPRIV.  


The typical method used to sequence current biologics, as well as map post translational modifications, is a combination of proteolytic digestion followed by LC/MS. Unfortunately, sometimes this method is not enough for complete identification of some post translational modifications. I am interested in the oxidation of Cys residues for the formation of disulfide bonds.  
The typical method used to sequence current biologics, as well as map post translational modifications, is a combination of proteolytic digestion followed by LC/MS. Unfortunately, sometimes this method is not enough for complete identification of some post translational modifications. I am interested in the oxidation of Cys residues for the formation of disulfide bonds.  
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<applet load='1ogs' size='[450,338]' frame='true' align='right'
<applet load='1ogs' size='[450,338]' frame='true' align='right'
caption='Disulfide Connectivity of GCase' scene='User:Adriana_Kita/Sandbox_1/Gcase_disulfide_1/2'/>
caption='Disulfide Connectivity of GCase ([[1ogs]])' scene='User:Adriana_Kita/Sandbox_1/Gcase_disulfide_1/2'/>
=== Disulfide Structure ===
=== Disulfide Structure ===


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'''Disulfide Connectivity:''' ''C4-C16 & C18-C23''
'''Disulfide Connectivity:''' ''C4-C16 & C18-C23''
==3D structures of beta-glucocerebrosidase==
[[Acid-beta-glucosidase]]
==Additional Resources==
For additional information, see: [[Metabolic Disorders]]
<br />


==Reference==
==Reference==
X-ray structure of human acid-beta-glucosidase, the defective enzyme in Gaucher disease., Dvir H, Harel M, McCarthy AA, Toker L, Silman I, Futerman AH, Sussman JL, EMBO Rep. 2003 Jul;4(7):704-9. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/12792654 12792654]
<references group="xtra"/>
[[Category: Glucosylceramidase]]
[[Category: Glucosylceramidase]]
[[Category: Single protein]]
[[Category: Single protein]]