Accelerate Your Biomolecule
and Bioassay Research
and Bioassay Research
sales@neoscientific.com
+1-888.733.6849
+1-617.299.7367 (Int’l)
+1-617.299.7367 (Int’l)
+1-888.733.6849
+1-617.299.7367 (Int’l)
+1-617.299.7367 (Int’l)
For quotations, please use our online quotation form, and you may also contact us by
sales@neoscientific.com
+1-888.733.6849
+1-617.299.7367 (Int’l)
+1-888.733.6849
+1-617.299.7367 (Int’l)
| Introduction | Branched-chain ?-keto acid dehydrogenase E1 component ? chain (BCKDHA) is a member of the BCKDHA family. The BCKD (branched-chain alpha-keto acid dehydrogenase) complex is an inner mitochondrial enzyme complex which catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. This complex consists of 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). Mutations in the BCKDHA gene result in maple syrup urine disease, type IA. |
| Synonyms | 2-oxoisovalerate dehydrogenase subunit alpha mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain, BCKDE1A, BCKDH E1-alpha, BCKDHA, MSU, MSUD1, OVD1A, FLJ45695. |
| Source | Escherichia Coli. |
| Physical Appearance | Sterile Filtered colorless solution. |
| Formulation | The BCKDHA solution (0.25 mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 5mM DTT, 30% glycerol and 0.2M NaCl. |
| Stability | Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles. |
| Amino Acid Sequence | MGSSHHHHHH SSGLVPRGSH MSSLDDKPQF PGASAEFIDK LEFIQPNVIS GIPIYRVMDR QGQIINPSED PHLPKEKVLK LYKSMTLLNT MDRILYESQR QGRISFYMTN YGEEGTHVGS AAALDNTDLV FGQYREAGVL MYRDYPLELF MAQCYGNISD LGKGRQMPVH YGCKERHFVT ISSPLATQIP QAVGAAYAAK RANANRVVIC YFGEGAASEG DAHAGFNFAA TL |
| Purity | Greater than 80.0% as determined by SDS-PAGE. |
| Usage | NeoScientific's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drµgs, agricultural or pesticidal products, food additives or household chemicals. |
N/A
N/A
Copyrights © 2014 NEO Group 245 First Street, 18th Floor, Cambridge MA 02142 888.733.6849
