Description
Product Name: | BCKDHA Polyclonal Antibody |
Product Code: | CAB21588 |
Reactivity: | Human, Mouse, Rat |
Applications: | Western blotting, Immunofluorescence |
Host Species: | Rabbit |
Purification Method: | Affinity purification |
Isotype: | IgG |
Reactivity: | Human, Mouse, Rat |
Tested Applications: | WB, IF/ICC, ELISA |
Key Applications: | Western blotting, Immunofluorescence |
Recommended Dilution: | WB 1:500-1:1000, IF/ICC 1:50-1:200 |
Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Positive Samples: | MCF7, 293T, Mouse kidney, Mouse liver, Rat liver, Rat kidney |
Cellular Location: | Mitochondrion matrix |
The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 276-445 of human BCKDHA (NP_000700.1). |
Sequence: | SEQYR GDGIA ARGPG YGIMS IRVDG NDVFA VYNAT KEARR RAVAE NQPFL IEAMT YRIGH HSTSD DSSAY RSVDE VNYWD KQDHP ISRLR HYLLS QGWWD EEQEK AWRKQ SRRKV MEAFE QAERK PKPNP NLLFS DVYQE MPAQL RKQQE SLARH LQTYG EHYPL DHFDK |
Synonyms: | MSU, MSUD1, OVD1A, BCKDE1A |
Calculated MW: | 50kDa |
Observed MW: | 50kDa |