The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
1/20. ab172730-Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.
1/1000 - 1/10000. Detects a band of approximately 34 kDa (predicted molecular weight: 34 kDa).
Involved in the catabolism of branched amino acids such as leucine.
Fibroblasts, liver and lymphoblasts.
Metabolic intermediate metabolism; (S)-3-hydroxy-3-methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1.
Involvement in disease
Defects in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD) [MIM:246450]; also known as hydroxymethylglutaricaciduria or HL deficiency. An autosomal recessive disease affecting ketogenesis and L-leucine catabolism. The disease usually appears in the first year of life after a fasting period and its clinical acute symptoms include vomiting, seizures, metabolic acidosis, hypoketotic hypoglycemia and lethargy. These symptoms sometimes progress to coma, with fatal outcome in some cases.