BCKDHB Rabbit Polyclonal Antibody
Frequently bought together (2)
Transient overexpression lysate of branched chain keto acid dehydrogenase E1, beta polypeptide (BCKDHB), nuclear gene encoding mitochondrial protein, transcript variant 2
USD 396.00
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Specifications
Product Data | |
Applications | WB |
Recommended Dilution | WB |
Reactivities | Human |
Host | Rabbit |
Isotype | IgG |
Clonality | Polyclonal |
Immunogen | The immunogen for Anti-BCKDHB antibody is: synthetic peptide directed towards the N-terminal region of Human BCKDHB. Synthetic peptide located within the following region: GFLHPAATVEDAAQRRQVAHFTFQPDPEPREYGQTQKMNLFQSVTSALDN |
Formulation | Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose. Note that this product is shipped as lyophilized powder to China customers. |
Purification | Affinity Purified |
Conjugation | Unconjugated |
Storage | Store at -20°C as received. |
Stability | Stable for 12 months from date of receipt. |
Predicted Protein Size | 23 kDa |
Gene Name | branched chain keto acid dehydrogenase E1, beta polypeptide |
Database Link | |
Background | Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein 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 transcript variants with different 3' non-coding regions, but encoding the same isoform. |
Synonyms | BCKDE1B; BCKDH E1-beta; E1B |
Note | Immunogen Sequence Homology: Human: 100%; Dog: 93%; Horse: 93%; Bovine: 93%; Rabbit: 93%; Pig: 86%; Rat: 79% |
Reference Data | |
Protein Pathways | Metabolic pathways, Valine, leucine and isoleucine degradation |
Documents
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*Delivery time may vary from web posted schedule. Occasional delays may occur due to unforeseen
complexities in the preparation of your product. International customers may expect an additional 1-2 weeks
in shipping.