Anti-HMGCL antibody [9F5AB9] (ab110295)
Key features and details
- Mouse monoclonal [9F5AB9] to HMGCL
- Suitable for: ICC/IF
- Reacts with: Human
- Isotype: IgG2b
Product nameAnti-HMGCL antibody [9F5AB9]
See all HMGCL primary antibodies
DescriptionMouse monoclonal [9F5AB9] to HMGCL
Tested applicationsSuitable for: ICC/IFmore details
Species reactivityReacts with: Human
Full length native protein (purified). This information is proprietary to Abcam and/or its suppliers.
- HeLa cells and human liver mitochondria.
This antibody clone is manufactured by Abcam. If you require a custom buffer formulation or conjugation for your experiments, please contact email@example.com.
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Product was previously marketed under the MitoSciences sub-brand.
Storage instructionsShipped at 4°C. Store at +4°C. Do Not Freeze.
Storage bufferpH: 7.5
Preservative: 0.02% Sodium azide
Constituent: HEPES buffered saline
Concentration information loading...
PurityAmmonium Sulphate Precipitation
Purification notesProduced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
Light chain typekappa
- Pathways and Processes
- Metabolic signaling pathways
- Lipid and lipoprotein metabolism
- Lipid metabolism
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab110295 in the following tested applications.
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 4 µg/ml.
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.
Cellular localizationMitochondrion matrix.
- Information by UniProt
- Entrez Gene: 3155 Human
- Omim: 246450 Human
- Omim: 613898 Human
- SwissProt: P35914 Human
- Unigene: 533444 Human
- 3 hydroxy 3 methylglutaryl CoA lyase antibody
- 3 hydroxy 3 methylglutaryl Coenzyme A lyase antibody
- 3 hydroxymethyl 3 methylglutaryl Coenzyme A lyase (hydroxymethylglutaricaciduria) antibody
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.
Datasheets and documents
ab110295 has not yet been referenced specifically in any publications.