BCKDHB pAb
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Product Description
Specifications
| Size | 20 µL, 100 µL |
| Estimated Lead Time | 1-2 weeks |
| Species | Mouse, Rat |
| Host Species | Rabbit |
| Accession Number | P21953 |
| Gene Id | 594 |
| Gene Symbols | BCKDHB |
| Protein Name / Synonyms | BCKDHB, BCKDE1B, BCKDH E1-beta, E1B, BCKDHE1-beta, branched chain keto acid dehydrogenase E1 subunit beta |
| Isotype | IgG |
| Purity | Affinity purification |
| Buffers | PBS with 0.02% sodium azide, 50% glycerol, pH7.3 |
| Recommended Applications | Western Blotting |
| Shipping Type | Blue ice |
| Storage | -20°C |
Background
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.
Dilutions
WB 1:500 - 1:2000
Format
Calculated MW
23kDa/43kDa
Observed MW
43kDa
Immunogen
Recombinant fusion protein containing a sequence corresponding to amino acids 183-392 of human BCKDHB (NP_898871.1).
Modification
Unmodified
Storage
Store at -20°. Avoid freeze / thaw cycles.
Images
Expiration:
12 months from the date of shipment when stored properly.