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Monday, April 28, 2014

Monday's Molecule #238

Last week's molecules (right) [Monday's Molecule #237] were α-D-glucopyranosyl-(1➝4)-β-D-glucopyranose, the β anomer of maltose and α-D-glucopyranosyl-(1➝6)-α-D-glucopyranose, the β anomer of isomaltose. The winner is Dean Bruce. A surprising number of people got it right.

This week's molecules (below) are related. They are cytoplasmic (PDB 2P8U left) and mitochondrial (PDB 2WYA right) versions of very important enzymes. The human versions are shown. Name the enzymes and the products bound in the active sites (complete common name of the product). Mutations in one of the genes coding for one of these enzymes cause a rare genetic disease. Which one? Why are there no known diseases associated with genetic defects in the gene for the other version of the enzyme?

Email your answer to me at: Monday's Molecule #238. The first one with the correct answer wins. I will only post the names of winners to avoid embarrassment. The winner will be treated to a free lunch.

There could be two winners. If the first correct answer isn't from an undergraduate student then I'll select a second winner from those undergraduates who post the correct answer. You will need to identify yourself as an undergraduate in order to win. (Put "undergraduate" at the bottom of your email message.)

In order to win you must post your correct name. Anonymous and pseudoanonymous commenters can't win the free lunch.

Winners will have to contact me by email to arrange a lunch date.

Comments are now open.

UPDATE: This one was pretty much a giveaway since I told you the PDB numbers of the two structures. Very few people submitted an answer ... I assume that's because you thought it was too easy. The winner is Dean Bruce (again). Here's his answer ...
... it is 3-hydroxy-3-methylglutarate-CoA (HMG-CoA) synthetase. HMG-CoA appears to be the only molecule in the active sites of the illustration. Acetoacetyl-CoA and Acetyl-CoA in a Claisen condensation (of the beta-carbonyl of acetoacetyl-CoA) form HMG-CoA.

The mitochondrial one is the isoform involved in metabolic pathology. The disease is called "mitochondrial HMG-CoA synthetase-2 deficiency" in the Online Mendelian Inheritance of Man.

Nov. 2009: Jason Oakley, Alex Ling
Oct. 17: Bill Chaney, Roger Fan
Oct. 24: DK
Oct. 31: Joseph C. Somody
Nov. 7: Jason Oakley
Nov. 15: Thomas Ferraro, Vipulan Vigneswaran
Nov. 21: Vipulan Vigneswaran (honorary mention to Raul A. Félix de Sousa)
Nov. 28: Philip Rodger
Dec. 5: 凌嘉誠 (Alex Ling)
Dec. 12: Bill Chaney
Dec. 19: Joseph C. Somody
Jan. 9: Dima Klenchin
Jan. 23: David Schuller
Jan. 30: Peter Monaghan
Feb. 7: Thomas Ferraro, Charles Motraghi
Feb. 13: Joseph C. Somody
March 5: Albi Celaj
March 12: Bill Chaney, Raul A. Félix de Sousa
March 19: no winner
March 26: John Runnels, Raul A. Félix de Sousa
April 2: Sean Ridout
April 9: no winner
April 16: Raul A. Félix de Sousa
April 23: Dima Klenchin, Deena Allan
April 30: Sean Ridout
May 7: Matt McFarlane
May 14: no winner
May 21: no winner
May 29: Mike Hamilton, Dmitri Tchigvintsev
June 4: Bill Chaney, Matt McFarlane
June 18: Raul A. Félix de Sousa
June 25: Raul A. Félix de Sousa
July 2: Raul A. Félix de Sousa
July 16: Sean Ridout, William Grecia
July 23: Raul A. Félix de Sousa
July 30: Bill Chaney and Raul A. Félix de Sousa
Aug. 7: Raul A. Félix de Sousa
Aug. 13: Matt McFarlane
Aug. 20: Stephen Spiro
Aug. 27: Raul A. Félix de Sousa
Sept. 3: Matt McFarlane
Sept. 10: Matt Talarico
Sept. 17: no winner
Sept. 24: Mikkel Rasmussen
Oct. 1: John Runnels
Oct. 8: Raúl Mancera
Oct. 15: Raul A. Félix de Sousa
Oct. 22: Mikkel Rasmussen
Nov. 12: Seth Kasowitz, Bill Gunn
Nov. 19: Michael Rasmussen
Dec. 4: Paul Clapham, Jacob Toth
Dec. 10: Jacob Toth
Dec. 17: Bill Chaney, Dima Klenchin, Bill Gunn
Jan. 14: Evey Salara
Jan. 21: Piotr Gasiorowski
March 11: Bill Gunn, River Jiang
March 18: Bill Gunn
April 8: Michael Florea
April 15: no winner
April 29: Anders Ernberg
May 6: Alex Ling, Michael Florea
May 13: Bill Chaney
June 24: Michael Florea
July 2: Matt McFarlane
July 8: no winner
July 15: Rosie Redfield, Thuc Quyen Huynh
July 22: Jacob Toth
July 29: Alex Ling, Matt McFarlane
August 5: Brian Shewchuk
Sept. 2: no winner
Sept. 9: Bill Chaney
Sept. 16: Zhimeng Yu
Sept. 23: Mark Sturtevant, Jacob Toth
Sept. 30: Susan Heaphy
Oct. 7: Piotr Gasiorowski, Jacob Troth
Oct. 14: Jean-Marc Neuhaus
Oct. 21: Jean-Marc Neuhaus
Oct. 28, 2013: Zhimeng Yu
Nov. 10, 2013: Caroline Josefsson, Andrew Wallace
Nov. 18, 2013: Dean Bruce, Ariel Gershon
Nov. 25, 2013: Jon Nuelle, Ariel Gershon
Dec. 2, 2013: Jean-Marc Neuhaus
Dec. 9, 2013: Bill Gunn
Dec. 16, 2013: Piotr Gasiorowski
Jan. 13, 2014: Tom Mueller
Jan. 20, 2014: Tommy Stuleanu
Jan. 27, 2014: Bill Gunn, Ariel Gershon
March 3, 2014: Keith Conover, Nevraj Kejiou
March 10, 2014: Philip Johnson
March 17, 2014: Jean-Marc Neuhaus
March 24, 2014: Frank Schmidt, Raul Félix de Sousa
March 31, 2014: Jon Binkley
April 7, 2014: no winner
April 21, 2014: Dean Bruce
April 28, 2014: Dean Bruce

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