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The disorder is the direct result of steric clash between the new phosphate group at Ser-264 and another serine, Ser-266, which destabilizes the hydrogen-bonding network that keeps the loops in place. Overall, this disorder results in the inactivation of the catalytic machinery. [13] The PDH complex can be regulated in a variety of conditions.
Pyruvate dehydrogenase (PDH) deficiency is a congenital degenerative metabolic disease resulting from a mutation of the pyruvate dehydrogenase complex (PDC) located on the X chromosome. While defects have been identified in all 3 enzymes of the complex, the E1-α subunit is predominantly the culprit.
Pymol-generated image of E1 subunit of pyruvate dehydrogenase complex in E. Coli. The E1 subunit, called the pyruvate dehydrogenase subunit, is either a homodimer (comprising two “α” chains, e.g. in Escherichia coli) or a heterotetramer of two different chains (two “α” and two “β” chains).
228026 Ensembl ENSG00000152256 ENSMUSG00000006494 UniProt Q15118 Q8BFP9 RefSeq (mRNA) NM_001278549 NM_002610 NM_172665 NM_001360002 RefSeq (protein) NP_001265478 NP_002601 NP_766253 NP_001346931 Location (UCSC) Chr 2: 172.56 – 172.61 Mb Chr 2: 71.7 – 71.73 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Pyruvate dehydrogenase lipoamide kinase isozyme 1, mitochondrial is an enzyme ...
The PDHB gene is responsible for the coding of the E1 beta subunit of the pyruvate dehydrogenase complex. The DLAT gene is responsible for the coding of the E2 subunit, and the PDP1 is responsible for producing the PDH phosphatase catalytic subunit that catalyzes PDH dephosphorylation. This dephosphorylation activates the complex.
The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 beta subunit.
381511 Ensembl ENSG00000164951 ENSMUSG00000049225 UniProt Q9P0J1 Q3UV70 RefSeq (mRNA) NM_001161778 NM_001161779 NM_001161780 NM_001161781 NM_018444 NM_001033453 NM_001098230 NM_001098231 NM_001290387 NM_001290391 RefSeq (protein) NP_001155251 NP_001155252 NP_001155253 NP_060914 NP_001028625 NP_001091700 NP_001091701 NP_001277316 NP_001277320 Location (UCSC) Chr 8: 93.86 – 93.93 Mb Chr 4: 11 ...
All these reactions are catalyzed by the E1, or pyruvate dehydrogenase, component of the PDH complex. The rest of the chemistry of the PDH complex is shown at the bottom of the figure. The acetyl group is transferred from reduced lipoamide to coenzyme A (CoA) by the activity of the E2, or dihydrolipoyl transacetylase, component of the complex ...