3-hydroxy-3-methylglutaryl coenzyme A lyase (HMG-CoA lyase, HMGCL)

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Function

  • ketogenesis & leucine catabolism; final step
(S)-3-hydroxy-3-methylglutaryl-CoA <--> acetyl-CoA + acetoacetate

Structure

Compartment

mitochondrial matrix

Expression

fibroblasts, liver & lymphoblasts

Pathology

-defects in HMGCL are the cause of hydroxymethylglutaricaciduria

More general terms

References

  1. Primary Hyperlipoproteinemias, Steiner & Shafrir (eds), McGraw Hill, NY, 1991, pg 14
  2. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=HMGCL
  3. UniProt http://www.uniprot.org/uniprot/P35914.html

Database