BCKDHB Rabbit Polyclonal Antibody (CAB10533)
- SKU:
- CAB10533
- Product Type:
- Antibody
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
Product Name: | BCKDHB Rabbit Polyclonal Antibody |
SKU: | CAB10533 |
Size: | 20uL, 100uL |
Isotype: | IgG |
Host Species: | Rabbit |
Reactivity: | Mouse,Rat |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 183-392 of human BCKDHB (NP_898871.1). |
Sequence: | RSPW GCVG HGAL YHSQ SPEA FFAH CPGI KVVI PRSP FQAK GLLL SCIE DKNP CIFF EPKI LYRA AAEE VPIE PYNI PLSQ AEVI QEGS DVTL VAWG TQVH VIRE VASM AKEK LGVS CEVI DLRT IIPW DVDT ICKS VIKT GRLL ISHE APLT GGFA SEIS STVQ EECF LNLE APIS RVCG YDTP FPHI FEPF YIPD KWKC YDAL RKMI NY |
Tested Applications: | WB ELISA |
Recommended Dilution: | WB,1:1000 - 1:5000 |
Synonyms: | E1B; BCKDE1B; BCKDH E1-beta; BCKDHB |
Positive Sample: | Mouse liver,Rat liver |
Conjugate: | Unconjugated |
Cellular Localization: | Mitochondrion matrix. |
Calculated MW: | 43kDa |
Observed MW: | 43kDa |
This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation and feeding problems. Alternative splicing at this locus results in multiple transcript variants.
Purification Method: | Affinity purification |
Gene ID: | 594 |
Storage Buffer: | Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3. |