Sandbox Reserved 1549

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== Disease ==
== Disease ==
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VLCDA clinical mutation can lead to disease state. VLCAD is used to break down very long-chain fatty acids, and they are found in food and body’s fat tissue(1). Fatty acids play a crucial role which provides energy for heart and muscle (1). Thus, VLCAD deficiency can cause severe neonatal cardiomyopathy and liver failure which occur mostly in adolescence or adulthood (2). In addition, if the body does not have sufficient amount of VLCAD, it would affect the metabolism of the body (1). There are several mutation sites that have been found. Mutation R429W is severe in childhood phenotype (2). Arg-429 on the helix K makes salt-bridge with Glu-384 on helix I (2). The enzyme is destabilized, and the salt bridge is broken due to the replacing charged residue with the bulky neutral residue2. Another mutation site is R416H which is found on the helix J2. Both sites, R429 and R416H, are located close the catalytic glutamate2. Site R416H has Arg-416 interacts with Asp 391 by making salt bridge and interacts with Gln-395 though hydrogen bond2. Mutation in R416H causes problem to the position of helix J2. Furthermore, forming salt bridge with the opposing monomer can affect the dimer interaction2.
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VLCDA clinical mutation can lead to disease state. VLCAD is used to break down very long-chain fatty acids, and they are found in food and body’s fat tissue(1). Fatty acids play a crucial role which provides energy for heart and muscle[[Link title]]. Thus, VLCAD deficiency can cause severe neonatal cardiomyopathy and liver failure which occur mostly in adolescence or adulthood (2). In addition, if the body does not have sufficient amount of VLCAD, it would affect the metabolism of the body (1). There are several mutation sites that have been found. Mutation R429W is severe in childhood phenotype (2). Arg-429 on the helix K makes salt-bridge with Glu-384 on helix I (2). The enzyme is destabilized, and the salt bridge is broken due to the replacing charged residue with the bulky neutral residue2. Another mutation site is R416H which is found on the helix J2. Both sites, R429 and R416H, are located close the catalytic glutamate2. Site R416H has Arg-416 interacts with Asp 391 by making salt bridge and interacts with Gln-395 though hydrogen bond2. Mutation in R416H causes problem to the position of helix J2. Furthermore, forming salt bridge with the opposing monomer can affect the dimer interaction2.
Deficient VLCAD affects 1 person in 40,000 to 120,000 people, thus, it is a very rare diseases1.
Deficient VLCAD affects 1 person in 40,000 to 120,000 people, thus, it is a very rare diseases1.

Revision as of 15:40, 30 April 2019

This Sandbox is Reserved from May 28 through July 01, 2019 for use in the course Advanced Biochemistry BCHM 4100 taught by Tom Gluick at the Georgia Gwinnett College. This reservation includes Sandbox Reserved 1544 through Sandbox Reserved 1555.
To get started:
  • Click the edit this page tab at the top. Save the page after each step, then edit it again.
  • Click the 3D button (when editing, above the wikitext box) to insert Jmol.
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More help: Help:Editing

Contents

A Very Long Chain Acyl-CoA Dehydrogenase

PDB ID 3B96

Drag the structure with the mouse to rotate

You may include any references to papers as in: the use of JSmol in Proteopedia [1] or to the article describing Jmol [2] to the rescue.

Function

Disease

VLCDA clinical mutation can lead to disease state. VLCAD is used to break down very long-chain fatty acids, and they are found in food and body’s fat tissue(1). Fatty acids play a crucial role which provides energy for heart and muscleLink title. Thus, VLCAD deficiency can cause severe neonatal cardiomyopathy and liver failure which occur mostly in adolescence or adulthood (2). In addition, if the body does not have sufficient amount of VLCAD, it would affect the metabolism of the body (1). There are several mutation sites that have been found. Mutation R429W is severe in childhood phenotype (2). Arg-429 on the helix K makes salt-bridge with Glu-384 on helix I (2). The enzyme is destabilized, and the salt bridge is broken due to the replacing charged residue with the bulky neutral residue2. Another mutation site is R416H which is found on the helix J2. Both sites, R429 and R416H, are located close the catalytic glutamate2. Site R416H has Arg-416 interacts with Asp 391 by making salt bridge and interacts with Gln-395 though hydrogen bond2. Mutation in R416H causes problem to the position of helix J2. Furthermore, forming salt bridge with the opposing monomer can affect the dimer interaction2.

Deficient VLCAD affects 1 person in 40,000 to 120,000 people, thus, it is a very rare diseases1.


Structural highlights

This is a sample scene created with SAT to by Group, and another to make of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.

</StructureSection>

References

  1. ↑ Hanson, R. M., Prilusky, J., Renjian, Z., Nakane, T. and Sussman, J. L. (2013), JSmol and the Next-Generation Web-Based Representation of 3D Molecular Structure as Applied to Proteopedia. Isr. J. Chem., 53:207-216. doi:http://dx.doi.org/10.1002/ijch.201300024
  2. ↑ Herraez A. Biomolecules in the computer: Jmol to the rescue. Biochem Mol Biol Educ. 2006 Jul;34(4):255-61. doi: 10.1002/bmb.2006.494034042644. PMID:21638687 doi:10.1002/bmb.2006.494034042644
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