存放说明Shipped at 4°C. Store at +4°C. Do Not Freeze.
存储溶液Preservative: 0.02% Sodium azide
Constituent: HEPES buffered saline
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纯度Ammonium Sulphate Precipitation
纯化说明Produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
- Pathways and Processes
- Metabolic signaling pathways
- Lipid and lipoprotein metabolism
- Lipid metabolism
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.
|ICC/IF||Use a concentration of 4 µg/ml.|
|In-Cell ELISA||Use a concentration of 1 µg/ml. (0.1 µg/well)|
|IP||Use at an assay dependent dilution.|
功能Involved in the catabolism of branched amino acids such as leucine.
组织特异性Fibroblasts, liver and lymphoblasts.
通路Metabolic intermediate metabolism; (S)-3-hydroxy-3-methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1.
疾病相关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.
序列相似性Belongs to the HMG-CoA lyase family.
- Information by UniProt
- 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.
ab110295 has not yet been referenced specifically in any publications.