369545
HMGCL (HL, 3-Hydroxymethyl-3-Methylglutaryl Coenzyme A Lyase, Hydroxymethylglutaricaciduria, Hydroxymethylglutaryl-CoA Lyase, Mitochondrial)
Size
£626.00
- SKU:
- 369545
- Application:
- WB
- Purity:
- Purified by Protein A and peptide affinity chromatography.
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Rabbit
- Immunogen:
- HMGCL (Met1~Leu325)
- Format:
- Supplied as a liquid in 0.01M PBS, pH 7.4, 0.05% Proclin-300, 50% glycerol.
- Extra Details:
- 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. Applications: Suitable for use in Western Blot. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Shipping Conditions:
- Blue Ice