Product Information
85948-1-PBS targets MCEE in WB, Indirect ELISA applications and shows reactivity with human, mouse, rat samples.
| Tested Reactivity | human, mouse, rat |
| Host / Isotype | Rabbit / IgG |
| Class | Recombinant |
| Type | Antibody |
| Immunogen |
CatNo: Ag9665 Product name: Recombinant human MCEE protein Source: e coli.-derived, PGEX-4T Tag: GST Domain: 1-176 aa of BC020825 Sequence: MARVLKAAAANAVGLFSRLQAPIPTVRASSTSQPLDQVTGSVWNLGRLNHVAIAVPDLEKAAAFYKNILGAQVSEAVPLPEHGVSVVFVNLGNTKMELLHPLGLDSPIAGFLQKNKAGGMHHICIEVDNINAAVMDLKKKKIRSLSEEVKIGAHGKPVIFLHPKDCGGVLVELEQA Predict reactive species |
| Full Name | methylmalonyl CoA epimerase |
| Calculated Molecular Weight | 176 aa, 19 kDa |
| Observed Molecular Weight | 17 kDa |
| GenBank Accession Number | BC020825 |
| Gene Symbol | MCEE |
| Gene ID (NCBI) | 84693 |
| RRID | AB_3744497 |
| Conjugate | Unconjugated |
| Form | Liquid |
| Purification Method | Protein A purification |
| UNIPROT ID | Q96PE7 |
| Storage Buffer | PBS only, pH 7.3. |
| Storage Conditions | Store at -80°C. |
Background Information
The MCEE protein (methylmalonyl-CoA difunctionalase) is a key metabolic enzyme located in the mitochondria. Its core function is to convert the (R)-methylmalonyl-CoA, which cannot be utilized by the downstream processes, into the (S)-configuration that can be recognized, thereby ensuring the normal progression of branched-chain amino acid and odd-chain fatty acid catabolism. The final product, succinyl-CoA, can enter the tricarboxylic acid cycle for energy production. If the gene encoding this protein undergoes mutation, it will lead to the accumulation of methylmalonyl-CoA and its derivatives, causing an autosomal recessive genetic disease - methylmalonic acidemia. Patients may experience severe metabolic disorders, neurotoxicity, and multi-organ damage. Therefore, MCEE plays a crucial role in maintaining energy metabolic homeostasis and preventing genetic metabolic diseases.
