Price range: $268.00 through $328.00
Background:
This gene encodes a mitochondrially localized enzyme that catalyzes the reversible formation of acetoacetyl-CoA from two molecules of acetyl-CoA. Defects in this gene are associated with 3-ketothiolase deficiency, an inborn error of isoleucine catabolism characterized by urinary excretion of 2-methyl-3-hydroxybutyric acid, 2-methylacetoacetic acid, tiglylglycine, and butanone. [provided by RefSeq, Feb 2009],
| Size | 50μL/100μL |
| Target | ACAT-1 |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC, IF, ELISA |
| MW(Calculated) | |
| MW(Observed) | 45kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-1:2000; IHC 1:100-1:300; ELISA 1:40000; IF 1:50-200 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | The antiserum was produced against synthesized peptide derived from human ACAT1. AA range:221-270 |
| Specificity | ACAT-1 Polyclonal Antibody detects endogenous levels of ACAT-1 protein. |
| Gene Name | ACAT1 |
| Protein Name | Acetyl-CoA acetyltransferase mitochondrial |
| Other Name | ACAT1; / ACAT; / MAT; / Acetyl-CoA acetyltransferase; / mitochondrial; / Acetoacetyl-CoA thiolase; / T2 |
| Species | Human |
| Gene ID | 38 |
| Uniprot | P24752, |
| Species.1 | Mouse |
| Gene ID.1 | 110446 |
| Uniprot.1 | Q8QZT1, |
| Species.2 | Rat |
| Gene ID.2 | 25014 |
| Uniprot.2 | P17764 |
| Cellular Localization | Mitochondrion . |
| Tissue Expression | Adipocyte,Brain,Fetal brain cortex, |
| Signaling_pathway | |
| Research Areas | >>Fatty acid degradation; / >>Valine, leucine and isoleucine degradation; / >>Lysine degradation; / >>Tryptophan metabolism; / >>Pyruvate metabolism; / >>Glyoxylate and dicarboxylate metabolism; / >>Butanoate metabolism; / >>Terpenoid backbone biosynthesis; / >>Metabolic pathways; / >>Carbon metabolism; / >>Fatty acid metabolism; / >>Fat digestion and absorption |
| Function | Catalytic activity:2 acetyl-CoA = CoA + acetoacetyl-CoA.,Disease:Defects in ACAT1 are a cause of 3-ketothiolase deficiency (3KTD) [MIM:203750]; also known as alpha-methylacetoaceticaciduria. 3KTD is an inborn error of isoleucine catabolism characterized by intermittent ketoacidotic attacks associated with unconsciousness. Some patients die during an attack or are mentally retarded. Urinary excretion of 2-methyl-3-hydroxybutyric acid, 2-methylacetoacetic acid, triglylglycine, butanone is increased. It seems likely that the severity of this disease correlates better with the environmental or acquired factors than with the ACAT1 genotype.,enzyme regulation:Activated by potassium ions, but not sodium ions.,Function:Plays a major role in ketone body metabolism.,similarity:Belongs to the thiolase family.,subunit:Homotetramer., |
