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 ELISA, Western Blot, Immunoprecipitation, Immunohistochemistry, Immunocytochemistry.
Other applications not tested.
Recommended Dilution:ELISA: 1:100-1:5000 Western Blot: 1:50-400Immunohistochemistry (frozen): 1:50-500Immunohistochemistry (paraffin): 1:10-100Immunocytochemistry: 1:50-500Optimal 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.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
