BCKDHA Rabbit Polyclonal Antibody (CAB16774)
- SKU:
- CAB16774
- Product Type:
- Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
Product Name: | BCKDHA Rabbit Polyclonal Antibody |
SKU: | CAB16774 |
Size: | 20uL, 100uL |
Isotype: | IgG |
Host Species: | Rabbit |
Reactivity: | Human,Mouse,Rat |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 276-445 of human BCKDHA (NP_000700.1). |
Sequence: | SEQY RGDG IAAR GPGY GIMS IRVD GNDV FAVY NATK EARR RAVA ENQP FLIE AMTY RIGH HSTS DDSS AYRS VDEV NYWD KQDH PISR LRHY LLSQ GWWD EEQE KAWR KQSR RKVM EAFE QAER KPKP NPNL LFSD VYQE MPAQ LRKQ QESL ARHL QTYG EHYP LDHF DK |
Tested Applications: | WB ELISA |
Recommended Dilution: | WB,1:1000 - 1:5000 |
Synonyms: | MSU; MSUD1; OVD1A; BCKDE1A; BCKDHA |
Positive Sample: | 293T,Mouse liver,Rat kidney |
Cellular Localization: | Mitochondrion matrix. |
Calculated MW: | 50kDa |
Observed MW: | 50kDa |
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.
Purification Method: | Affinity purification |
Gene ID: | 593 |
Storage Buffer: | Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3. |