The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 4 µg/ml.
Use a concentration of 1 µg/ml. (0.1 µg/well)
Use at an assay dependent dilution.
FunctionInvolved in the catabolism of branched amino acids such as leucine.
Tissue specificityFibroblasts, liver and lymphoblasts.
PathwayMetabolic intermediate metabolism; (S)-3-hydroxy-3-methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1.
Involvement in diseaseDefects 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.
Sequence similaritiesBelongs to the HMG-CoA lyase family.
Immunocytochemistry image of ab110295 stained Human HeLa cells. The cells were paraformaldehyde fixed (4%, 20 minutes) and Triton X-100 permeabilized (0.1%, 15 minutes). The cells were incubated with ab110295 at 5 µg/ml for 2 hours at room temperature or over night at 4°C. The secondary antibody was (green) Alexa Fluor® 488 goat anti-mouse IgG (H+L) used at a 1/1000 dilution for 1 hour. 10% Goat serum was used as the blocking agent for all blocking steps. Target protein locates mainly in mitochondria.
References for Anti-HMGCL antibody [9F5AB9] (ab110295)
has not yet been referenced specifically in any publications.
Publishing research using ab110295? Please let us know so that we can cite the reference in this datasheet.
Concentration of lot no. is
Concentration not available for this lot.
Find concentration of your lot:
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"