3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL)
HL; Hydroxymethylglutaricaciduria; Hydroxymethylglutaryl-CoA lyase, mitochondrial
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3-Hydroxy-3-methylglutaryl coenzyme A lyase is a homodimeric mitochondrial matrix enzyme that catalyzes the cleavage of HMG-CoA to acetoacetic acid and acetyl-CoA, the last step of both ketogenesis and leucine catabolism. A shortage of glucose-sparing ketone bodies normally produced during fasting was thought to be responsible for the hypoglycemia which characterizes this metabolic defect. The absence of ketonuria in this disorder is a direct consequence of the metabolic lesion. HMG-CoA lyase is involved in ketogenesis, and the patient with the deficiency is compromised in the ability to generate ketone bodies. Both parents had reduced levels of HMG-CoA lyase in leukocytes. Clinically, deficiency of the enzyme results in metabolic acidosis and hypoglycemia.
Organism species: Homo sapiens (Human)
- Recombinant protein RPD685Hu01 Recombinant 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) In Stock
- Polyclonal antibody PAD685Hu01 Polyclonal Antibody to 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) In Stock
- Customized Service n/a CLIA Kit for 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) CLIA Kit Customized Service Offer
- Customized Service n/a ELISA Kit for 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) ELISA Kit Customized Service Offer
Organism species: Mus musculus (Mouse)
- Recombinant protein RPD685Mu01 Recombinant 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) In Stock
- Polyclonal antibody PAD685Mu01 Polyclonal Antibody to 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) In Stock
- Customized Service n/a CLIA Kit for 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) CLIA Kit Customized Service Offer
- Customized Service n/a ELISA Kit for 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) ELISA Kit Customized Service Offer
Organism species: Rattus norvegicus (Rat)
- Customized Service n/a Recombinant 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) Recombinant Protein Customized Service Offer
- Customized Service n/a Monoclonal Antibody to 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) Monoclonal Antibody Customized Service Offer
- Customized Service n/a Polyclonal Antibody to 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) Polyclonal Antibody Customized Service Offer
- Customized Service n/a CLIA Kit for 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) CLIA Kit Customized Service Offer
- Customized Service n/a ELISA Kit for 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase (HMGCL) ELISA Kit Customized Service Offer