BCKDHB Monoclonal Antibody (CAB24618)
- SKU:
- CAB24618
- Product Type:
- Antibody
- Antibody Type:
- Monoclonal Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- WB
- Applications:
- IHC
Description
| Product Name: | BCKDHB Monoclonal Antibody |
| Product Code: | CAB24618 |
| Reactivity: | Human, Mouse |
| Applications: | Western blotting, Immunohistochemistry |
| Host Species: | Rabbit |
| Purification Method: | Affinity purification |
| Isotype: | IgG |
| Clone No: | ARC62319 |
| Reactivity: | Human, Mouse |
| Tested Applications: | WB, IHC-P, ELISA |
| Key Applications: | Western blotting, Immunohistochemistry |
| Recommended Dilution: | WB 1:1000-1:5000, IHC-P 1:50-1:200 |
| Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles.Buffer: PBS with 0.05% proclin300, 0.05% BSA, 50% glycerol, pH7.3. |
| Positive Samples: | HeLa, 293F |
| Cellular Location: | Mitochondrion matrix |
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.
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 51-392 of human BCKDHB (NP_898871.1). |
| Sequence: | VAHFT FQPDP EPREY GQTQK MNLFQ SVTSA LDNSL AKDPT AVIFG EDVAF GGVFR CTVGL RDKYG KDRVF NTPLC EQGIV GFGIG IAVTG ATAIA EIQFA DYIFP AFDQI VNEAA KYRYR SGDLF NCGSL TIRSP WGCVG HGALY HSQSP EAFFA HCPGI KVVIP RSPFQ AKGLL LSCIE DKNPC IFFEP KILYR AAAEE VPIEP YNIPL SQAEV IQEGS DVTLV AWGTQ VHVIR EVASM AKEKL GVSCE VIDLR TIIPW DVDTI CKSVI KTGRL LISHE APLTG GFASE ISSTV QEECF LNLEA PISRV CGYDT PFPHI FEPFY IPDKW KCYDA LRKMI NY |
| Synonyms: | E1B, BCKDE1B, BCKDH E1-beta |
| Calculated MW: | 43kDa |
| Observed MW: | 37kDa |
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