Simple Introduction

Pyruvate dehydrogenase complex

Consists of 3 subunit enzyme:

Enzyme	Abbreviated	Prosthetic Group
Pyruvate Dehydrogenase	E1	Thiamine pyrophosphate (TPP)
Dihydrolipoyl Transacetylase	E2	Lipoamide
Dihydrolipoyl Dehydrogenase	E3	FAD

How is it linked to the disease?

Pyruvate dehydrogenase complex is required to convert pyruvate(produced from glycolysis) to acetyl coA. Acetyl CoA then act as a precursor to undergo Krebs Cycle.

In aerobic respiration, Krebs cycle and Glycolysis are IMPORTANT because they produce ATP(chemical energy) from our diet(glucose)

What is Pyruvate dehydrogenase complex deficiency (PDHA)?

Pyruvate dehydrogenase complex deficiency (PDHA) is a genetic disorder that results in a malfunctioning of the Krebs, or tricarboxylic acid (TCA), cycle. It is sex-linked and appears to be a dominant trait.

Further details are shown in the video below:

Source: Diwan, Joyce J. Pyruvate Dehydrogenase & Krebs Cycle. 1998-2007. Web. <http://www.rpi.edu/dept/bcbp/molbiochem/MBWeb/mb1/part2/krebs.htm>.

Videos featuring patients suffering from the disease

Interesting article on PDH structure

"Massive Enzyme Footballs Control Sugar Metabolism

ScienceDaily (July 18, 2011) — Neutron scattering has revealed how massive enzyme complexes inside cells might determine whether sugar is burnt for energy or stored as fat. The findings promise to improve understanding of diabetes and a range of metabolic diseases."

Read the full article here:  http://www.sciencedaily.com/releases/2011/07/110718091823.htm

Institut Laue-Langevin (ILL) (2011, July 18). Massive enzyme footballs control sugar metabolism. ScienceDaily. Retrieved July 9, 2012, from http://www.sciencedaily.com­/releases/2011/07/110718091823.htm