| Catalog# |
CF46 |
| Source |
E.coli |
| Description |
Recombinant Human Medium-Chain Specific Acyl-CoA Dehydrogenase Mitochondrial/ACADM is produced with our E. coli expression system. The target protein is expressed with sequence (Lys26-Asn421) of Human ACADM fused with a 6His tag at the N-terminus. |
| Names |
Medium-Chain Specific Acyl-CoA Dehydrogenase Mitochondrial, MCAD, ACADM |
| Accession # |
P11310 |
| Formulation |
Supplied as a 0.2 μm filtered solution of 20mM Tris, 0.1M NaCl, 20% Glycerol, pH 8.5 |
| Shipping |
The product is shipped on dry ice/ice packs. |
| Storage |
Store at < -20°C, stable for 6 months after receipt.
Please minimize freeze-thaw cycles. |
| Purity |
Greater than 95% as determined by SEC-HPLC and reducing SDS-PAGE. |
| Endotoxin |
Less than 0.1 ng/μg (1 IEU/μg). |
| Amino Acid Sequence |
MGSSHHHHHHSSGLVPRGSHMKANRQREPGLGFSFEFTEQQKEFQATARKFAREEIIPVAAEYDK TGEYPVPLIRRAWELGLMNTHIPENCGGLGLGTFDACLISEELAYGCTGVQTAIEGNSLGQMPII IAGNDQQKKKYLGRMTEEPLMCAYCVTEPGAGSDVAGIKTKAEKKGDEYIINGQKMWITNGGKAN WYFLLARSDPDPKAPANKAFTGFIVEADTPGIQIGRKELNMGQRCSDTRGIVFEDVKVPKENVLI GDGAGFKVAMGAFDKTRPVVAAGAVGLAQRALDEATKYALERKTFGKLLVEHQAISFMLAEMAMK VELARMSYQRAAWEVDSGRRNTYYASIAKAFAGDIANQLATDAVQILGGNGFNTEYPVEKLMRDA KIYQIYEGTSQIQRLIVAREHIDKYKN
|
| Background |
Medium-Chain Specific Acyl-CoA Dehydrogenase (ACADM) is a mitochondrial fatty acid beta-oxidation that belongs to the acyl-CoA dehydrogenase family. ACADM is a homotetramer enzyme that catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. ACADM is specific for acyl chain lengths of 4 to 16. It is essential for converting these particular fatty acids to energy, especially during fasting periods. Defects in ACADM cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death. |
